British Journal of Dermatology

July 2007 - Vol. 157 Issue s1 Page1-138

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Foreword pages iv–iv

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Disclaimer pages vi–vi

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Guest Speakers pages xx–xx

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Main Plenary Sessions

Main Plenary Sessions: Summaries of Papers pages 1–9

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Registrars’ Symposium pages 10–13

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Clinicopathological Cases

Clinicopathological Cases: Summaries of Papers pages 14–22

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Bristol Cup Posters

Bristol Cup Posters: Summaries of Posters pages 23–73

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Historical Archive Symposium

Historical Archive Symposium: Summaries of Papers pages 74–80

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Contact Dermatitis

British Society: Summaries of Papers pages 81–93

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Dermatopathology

British Society for Dermatopathology: Summary of Papers pages 94–105

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Dermatological Surgery

British Society for Dermatological Surgery: Summaries of Papers pages 106–118

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Society for Paediatric Dermatology

British Society for Paediatric Dermatology: Summaries of Papers pages 119–131

Abstracts for the British Association of Dermatologists 87th Annual Meeting Birmingham, U.K. 10-13 July 2007

Photodermatology

British Photodermatology Group: Summaries of Papers pages 132–138

Foreword

This supplement contains summaries of the papers and posters to be presented at the 87th Annual Meeting of the British Association of Dermatologists, to be held at the Birmingham International Convention Centre, U.K., 10–13 July 2007. Also included are summaries of the papers to be presented at the meetings of the following groups: British Contact Dermatitis Society, British Society for Dermatopathology, British Society for Dermatological Surgery, British Society for Paediatric Dermatology, British Photodermatology Group and Historical Archive Collection.

J.S.C. English Editor Disclaimer

This abstract book has been produced using author-supplied copy. Editing has been restricted to some corrections of spelling and style where appropriate. No responsibility is assumed for any claims, instructions, methods or drug dosages contained in the abstracts: it is recommended that these are verified independently.

Standard numbering of abstracts

The abstracts have been numbered following their order of presentation within each session. British Association of Dermatologists Annual Meeting, 2007, Birmingham

Guest Speakers Venue

Tuesday 10th July 2007 09:30–10:15 Prosser-White Oration Hall 11 Dr David Basketter (London, U.K.) Contact dermatitis and industry: twenty-five years on a tightrope 11:00–11:20 Update Lecture Hall 1 Dr Peter Colloby (Birmingham, U.K.) Histopathological aspects of Mohs’ surgery 11:00–11:20 Invited Neil Smith Dermatopathology Update Lecture Hall 11 Dr Gerald Saldanha (Leicester, U.K.) Histopathological aspects of Mohs’ surgery 15:20–15:50 Guest Speaker Hall 1 Dr Jon Hanifin (Oregon, U.S.A.) today and tomorrow 17:00–17:30 Political Update Hall 1 Dr Robin Graham-Brown (Leicester, U.K.) Care closer to home: where are we now?

Wednesday 11th July 2007 Venue 12:30–13:00 Guest Speaker Hall 1 Professor Francisco Kerdel (Miami, U.S.A.) Medical dermatology: new treatment for TEN and hidradenitis suppurativa 14:50–15:30 British Society for Paediatric Dermatology – Guest Speaker Hall 6 Professor Heiko Traupe (Mu¨nster, Germany) The ichthyoses: advances in molecular pathology and clinical management

Thursday 12th July 2007 Venue 12:15–13:00 Arthur Rook Oration Hall 1 Professor Irene Leigh (Dundee, U.K.) Translational research in dermatology 13:00–13:30 Historical Collection Symposium Hall 9 Professor Martin Black (London, U.K.) The history of St John’s Hospital for Diseases of the Skin: tales of the founder to the present day 14:00–14:35 British Photodermatology Group Hall 11 Dr Susan Walker (London, U.K.) The beneficial effects of UVR-induced immunosuppression 14:35–15:10 British Photodermatology Group Hall 1 Professor Mark Birch-Machin (Newcastle, U.K.) Sunburnt DNA: how mitochondria record UV exposure of the skin

Friday 13th July 2007 Venue 10:00–10:30 Guest Speaker Hall 1 Professor Martin Cook (Guildford, U.K.) pathology: essential knowledge for the dermatologist

The Authors xx Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): xx Main Plenary Sessions: Summaries of Papers

O-1 temperate summers may encourage outdoor bathing, making Cercarial dermatitis in the U.K. the presentation of CD more likely in the U.K.. S. Fraser, S. Allan and S. A. Holme Victoria Hospital, Kirkcaldy, U.K. Swimmer’s itch or cercarial dermatitis (CD) is an immuno- O-2 logical reaction to free-swimming parasites released from Dermatological workload: primary versus secondary freshwater snails. Shortly after leaving the infested water, care affected bathers develop a pruritic eruption which is gener- O.A. Kerr, E.C. Benton,* J.J. Walker, ally self-limiting. Although reported in continental Europe, R.D. Aldridge* and M.J. Tidman* North America and Asia, the condition has not previously Welsh Institute of Dermatology University Hospital of Wales, Cardiff, U.K.; been recorded in the U.K.. We report an outbreak of CD at *Dermatology Department Royal Infirmary of Edinburgh, Edinburgh, U.K. a local country park in July 2006. During hot weather, and Research Unit in Health, Behaviour and Change Royal Infirmary of bathing was popular in the loch, and four girls aged Edinburgh, Edinburgh, U.K. between 4 and 8 years presented with an itchy on The aims of this study were to estimate the burden of derma- their limbs and torso after paddling. Dermatological examin- tological disease in Primary Care, to record the diagnostic ation revealed an excoriated maculopapular eruption on each spectrum compared with that seen in hospital clinics, to evalu- girl’s limbs and torso, which was urticated in places. Treat- ate Primary Care management of frequently attending patients ment with mild topical corticosteroid, emollient and oral and to assess the reasons for referrals to hospital. Thirteen antihistamine provided symptomatic relief and the rash general medical practices serving 100 000 patients partici- resolved within 10 days in each case. The local Public pated. For a 2-week period during 2004–05, general practi- Health Department was alerted to nine further individuals tioners (GP) and practice nurses recorded all patients seen with a similar eruption who had bathed at the same loca- with a dermatological complaint. Subsequently a research tion. Analysis of a sample of molluscs from the water registrar extracted data from the medical records of these found evidence of cercarial infestation in 10% of 30 Ram- patients. Records from a control sample were examined to shorn snails collected. Warning notices were erected around assess compliance of the GPs. All patients who had consulted the shallow water to discourage bathing and the feeding of with a skin complaint on three or more occasions in the pre- water birds. CD is caused by the larvae of avian flukes vious year (frequent attenders) were invited to see a consult- (Trematoda) which have a complex life cycle (Kola´rˇova´ L, ant for evaluation of diagnosis, management plan and the Skirnisson K, Hora´k P. Schistosome cercariae as the causative need for referral to Secondary Care. In November 2005, hos- agent of swimmer’s itch in Iceland. J Helminthol 1999; 73: pital dermatology consultations in a region serving a popula- 215–20). Water birds are the primary host and the ciliated tion of 1Æ2 million were recorded by medical staff to assess larvae (miracidiae) hatch from eggs in the birds’ faeces. the diagnostic spectrum of new and return patients and the The miracidiae penetrate aquatic snails and develop into reasons for referral. There was wide variation between prac- sporocysts. Hundreds of motile larvae (cercariae) are then tices in the proportion of skin-related consultations (4–19%), released into the water by the snail, which seek aquatic with low recorders showing evidence of poor study compli- birds to restart the life cycle. Humans are affected due to ance. In a single practice where crosschecks showed no evi- the cercaria’s ability to penetrate mammalian stratum cor- dence of under-recording, the proportion of dermatological neum resulting in an inflammatory response, although the consultations was 15% (CI 13Æ8–18Æ3%). Of 720 dermato- fluke’s life cycle is arrested. The differential diagnosis logical consultations, the most frequent diagnoses were includes insect bites, human schistosomiasis, contact derma- eczema 22%, infections or infestation 20%, benign tumours titis, and sea bather’s eruption. The diagnosis of CD 11%, 5%, 5%, 4% and skin cancer 2%. depends on clinical features and history of exposure to This contrasts with the diagnoses of the 4844 hospital clinic infested water. Confirming the presence of cercariae in a consultations: benign tumours 24%, skin cancer 16%, eczema body of water can prove difficult as they die shortly after 16%, psoriasis 11%, acne 7%, warts 3% and infection or release, and often a low percentage of snails are infested. infestation 2%. Primary Care frequent attenders comprised Although individual cases are self-limiting, an outbreak pre- 239 of 720 patients (33%). Of these 239, 118 were examined sents a significant public health problem. Molluscicides have by a consultant. Several recurring management deficiencies been employed, along with mechanical disruption of the were noted. Referral rates from Primary to Secondary Care snail’s habitat, but these methods are only partially success- during the study period varied between practices from 0 to ful at interrupting the parasite’s life cycle. Increasingly 6%. The hospital audit in November 2005 recorded data on

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 1 2 Main Plenary Sessions

2049 new referrals: 57% of these were for a diagnostic opin- established. Further study of new to follow up ratios is ion and 38% for management advice. In conclusion, dermato- required. The large disparity between the current referral logical complaints accounted for 1 in 7 consultations in rates to secondary care departments and that proposed in the Primary Care. The spectrum of disease differed from that seen White Paper suggests that this figure may be unachievable in Secondary Care with GPs seeing few skin cancers compared and should be revisited. with hospital dermatologists. Over half of GP referrals are made for diagnostic purposes. Training for Primary Care should perhaps focus on the diagnosis and management of O-4 those conditions most often seen by GPs. Genetic variation across the methotrexate metabolic pathway and its influence upon treatment outcomes in patients with psoriasis O-3 R.B. Warren, R. Smith,* E. Campalani, Care closer to home and dermatology services: how J.N.W.N. Barker, C. Smith, J. Worthington* and are we doing? C.E.M. Griffiths J.K. Schofield, D. Shuttleworth, S. George and The Dermatology Centre, The University of Manchester, Manchester M6 R.A.C. Graham-Brown 8HD, U.K.; *Epidemiology Unit, The University of Manchester, Manchester Department of Health Care Closer to Home Group, London, U.K. M13 9PT, U.K. and Skin Therapy Research Unit, St John’s Institute of The White Paper ‘Our Health, our care, our say: a new Dermatology, Kings College London, London SE1 7EH, U.K. direction for community services’, (Chapter 6, Care closer to Background methotrexate (MTX), a first-line systemic ther- home. Department of Health 2006) makes specific recom- apy for moderate-to-severe psoriasis, is limited in its use by mendations in respect of shifting care within particular spe- unpredictable efficacy and toxicity. Preliminary studies in cialities into community settings. The commitments for rheumatoid arthritis and psoriasis patients treated with MTX dermatology include the delivery of up to 30% of specialist have suggested that genetic variation within the MTX meta- services in community settings, a ratio of new to follow-up bolic pathway may be predictive of treatment-related out- consultations of 1:1Æ53, the provision of care for long term comes. However such studies, to date, have looked at skin conditions (e.g. psoriasis and eczema) in community isolated single nucleotide polymorphisms (SNPs) within only settings, the development of General Practitioners and Practi- a few of the relevant genes in the MTX metabolic pathway. tioners with a Special Interest (GPwSI and PwSI) services and Results are variable with little replication. Furthermore, a referral rate of 2Æ89 per 1000 population to secondary care recent advances both in our understanding of the mechan- services. The aim of this study, which was completed to ism of action of MTX and in the publication of large num- inform the Department of Health Dermatology Care Closer bers of SNPs allows: (i) a logical approach to candidate to Home stakeholder group, was to review current dermatol- gene selection; and (ii) a comprehensive study of how gen- ogy service provision in this context. A questionnaire was etic variation within the MTX metabolic pathway may alter devised by the group and circulated widely to members of treatment outcomes in psoriasis patients. Patients and the British Association of Dermatologists (BAD), British Der- methods: DNA was collected from 378 patients with psoria- matology Nursing Group, the Primary Care Dermatology sis who had been treated with MTX. Phenotype data was Society and Primary Care Trust Chief Executives. Responses available as to efficacy, with polarization into two groups: were returned to and evaluated by one of the authors. A responders and non-responders. Liver and gastrointestinal total of 96 responses were received of which 57 were from toxicities were recorded also. Haplotype tagging of SNPs hospital secondary care dermatology departments. This report from the phase II HapMap data were selected from two reviews only these responses. Forty one (72%) of respondent MTX efflux genes, ABCC1 and ABCG2; a key enzyme departments are offering services in community settings, involved in folate metabolism, MTHFR; and two adenosine most (31 of 41) in community or nonacute hospitals. These receptors, ADORA A1 and ADORA A2A, thought to mediate services provide care for a mean of 23% of total caseload the immune-modulating and anti-inflammatory effects of (range 5–100). The new to follow-up ratio (1:1Æ53) was MTX. Genotyping was undertaken using a mass spectromet- met in 57% of departments. The mean new to follow-up ric method and analysis performed with the statistical soft- ratio across all responses was 1:1Æ58 (range 0Æ8–2Æ9). Care ware package STATA version 8Æ2. Results: A total of 91 in community settings for patients with long term skin con- SNPs in five genes were analysed. One nonsynonymous SNP ditions is offered by 74% of departments. Specialist derma- in exon 28 of ABCC1, rs28364006, was significantly (P = tology nurses are found in 60% of departments and 68% 0Æ03, trend test P =0Æ03) associated with MTX responders. have GPwSIs working in the local health community (72% A further SNP in ABCC1 (rs246240) was associated with integrated, 28% not). The referral rate per 1000 population the development of any adverse event (P =0Æ018, trend varied between 10 and 21Æ8. This study demonstrates that test P =0Æ016). No SNPs in ABCG2 were associated with many patients with skin disease already receive their care any form of treatment outcome in psoriasis patients treated from specialists in community settings, including those with with MTX. One SNP rs5760410 (ADORA A2A) was signifi- chronic skin disease. GPwSI and nurse services are well cantly associated (P =0Æ046, trend test P =0Æ0226) with

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 Main Plenary Sessions 3

MTX responders. Two known functional SNPs in MTHFR, O-6 which have been shown to alter both the response to and Diagnosis and management of cutaneous toxicity from MTX in rheumatoid arthritis patients, were side-effects in patients receiving treatment not associated with outcome to MTX in psoriasis patients. based on epidermal growth factor blockade Conclusion: these data indicate that SNPs in the ABC family M. O’Connell, P.S. Mortimer,* A. Melcher and of efflux transporters (ABCC1) and adenosine receptor A.M. Layton (ADORA2A) may be of predictive value when identifying Department of Dermatology, Harrogate and District NHS Foundation Trust, psoriasis patients suitable for MTX therapy. Ideally these Harrogate, N. Yorkshire, U.K.; *The Royal Marsden NHS Foundation Trust results should be validated in a prospective trial. and St George’s Hospital, London, U.K. and Cancer Research U.K. Oncology Unit, St James’ Hospital, Leeds, Yorkshire, U.K. Aberrant epidermal growth factor (EGF) signalling plays a piv- otal role in the evolution of many cancers. Thus, it is not surpri- O-5 sing that many of the newer target based cancer therapies have Fine mapping of susceptibility genes for atopic focused on EGF signal blockade. This targeted approach has led dermatitis in the epidermal differentiation complex to a higher therapeutic index and decreased incidence of toxicity on chromosome 1q21 when compared with standard anticancer drugs. With increas- N. Morar, P. Edster,* T. Street,* S. Weidinger, ing use of these agents, it has become clear that there is an in- W-L. Di, A. Dixon,* M. Taylor,* R. Holt,* J. Broxholme,* N. Kloop,§ N. Novak,– creased incidence of cutaneous side-effects. The most common A. Bockelbrinck, J. Ragoussis,* T. Illig,§ J. Harper, adverse reaction is an acneiform skin eruption that occurs in W. Cookson and M. Moffatt 35–100% of patients. Approximately 10% of these patients Molecular Genetics, NHLI, Imperial College London, London, U.K.; demonstrate the most severe or Grade 3 type response. Other *Wellcome Trust Centre for Human Genetics, Oxford, U.K.; Department common cutaneous manifestations associated with EGFR block- of Dermatology and Allergy, Techincal University, Munich, Germany; ade include xerosis that affects approximately one third of Great Ormond Street Hospital and Institute of Child Health, London, U.K.; patients and which is seen in about 10% of cases §GSF-National Research Centre for Environment and Health, Munich, (Segaert S, Tabernero J, Chosidow O et al. The management of Germany and –Department of Dermatology and Allergy, Bonn, Germany skin reactions in cancer patients receiving epidermal growth fac- Atopic dermatitis (AD) results from the interaction between tor receptor targeted therapies. J Dtsch Dermatol Ges 2005: 3; 599– strong genetic and environmental factors. Genome screens 606). This is not surprising given the central role EGF plays in have identified chromosome 1q21 as being linked to AD. This follicular homeostasis, keratinocyte signalling and in mediating region harbours the epidermal differentiation complex (EDC). the inflammatory response of skin. However, an unexpected ob- The EDC comprises several genes and gene families that are servation is that the severity of these cutaneous sequelae relates expressed during terminal differentiation of the . positively to their antitumour effect and patient survival (Wyatt Recently mutations in one of the EDC genes, filaggrin (FLG), AJ, Leonard GD, Sachs Dana L. Cutaneous reactions to chemo- have been associated with AD. We genotyped these mutations therapy and their management. Am J Clin Dermatol 2006; 7: in two panels of families, ECZ1 and MRCE, recruited through 45–63).n Dermatologists are faced with diagnosing and man- a child with severe AD. The panels totalled 426 families con- aging these drug induced skin manifestations, which often have taining 990 affected and unaffected children. Using familial a significant effect on the patient’s quality of life and can make tests of association we replicated the FLG associations (P = patients unwilling to comply with therapy. We report several 0.0001). We reanalysed the data for the previously reported clinical cases describing the common cutaneous adverse effects highly linked marker D1S498 on chromosome 1q21. For AD, associated with EGFR blockade, with treatment options on how the evidence of linkage to the markers was a total LOD score best to manage these problems. of 3.57. This fell to 2.03 after accounting for the FLG muta- tions, indicating the presence of other genetic variants influen- cing AD at this locus. We systematically resequenced genes O-7 and examined public databases to identify single nucleotide Photoprotective behaviour and sunscreen use: the polymorphisms (SNPs) within the EDC. Initially 100 markers effect on vitamin D levels in cutaneous were genotyped in the ECZ1 panel using Sequenom erythematosus (San Diego, CA, U.S.A.) technology. Potential associations C. Cusack, B. Murray and G.M. Murphy (P £ 0.01) were confirmed in the MRCE panel. Regions of Beaumont Hospital, Dublin, Ireland confirmed association were saturated with a further 30 SNP Vitamin D deficiency has been recognised in several population markers. We found two regions of highly replicated genetic groups due to insufficient sun exposure, prolonged use of sun- association (P =7Æ9E-05 and P =0Æ001) within the EDC. screens or inadequate dietary intake. A theoretical risk of vita- Neither marker was in linkage disequilibrium with each other min D deficiency secondary to photoprotection which also indicating that they have independent genetic effects. The includes regular sunscreen application led us to investigate if results indicate that the genes of the EDC may have multiple patients with photo-aggravated cutaneous lupus erythematosus effects on epidermal immunity and barrier formation. are deficient in vitamin D. Approval was obtained from the

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 4 Main Plenary Sessions

Hospital Ethics Committee and patients with biopsy proven patients (six males and nine females; median age 42 years) and cutaneous lupus erythematosus who attend the outpatient 61 immunocompetent patients (30 males and 31 females; med- department were included in this study. Their vitamin D levels ian age 35 years). All patients had received at least two forms of were measured during June to September 2006 using the conventional therapy before being considered for DCP. Patients universally accepted 25(OH) vitamin D assay. Patients also com- were sensitized and then treated with increasing concentrations pleted a questionnaire which recorded the Fitzpatrick skin type of DCP. The median duration of warts was four years prior to and ingestion of vitamin supplements as well as a detailed analy- commencing DCP treatment which was administered once per sis of photoprotection. A total of 50 patients were included in month. The median duration of treatment in the immunocom- this study: 9 males and 41 females with ages ranging from 22 to promised group was significantly greater than in the immuno- 76 years. All had skin type I–III apart from two individuals with competent patients (9 vs. 6 months respectively, P = 003). skin type IV. The diet of 43% was supplemented with daily vita- However, there was no statistically significant difference min D preparations, 71% reported avoidance of sunlight and between immunocompromised and immunocompetent patients 51% used sunscreens daily. A mean vitamin D level of 66Æ3 with regard to the maximum strength of DCP required (4% vs. nmol L–1 was recorded (SD = 24Æ9), normal laboratory refer- 6% respectively), and the proportion of patients achieving com- ence levels >70 nmol L–1 during the summer period. Critically plete resolution of their warts (40 of 61 (66%) vs. 9 of 15 low levels (<25 nmol L–1) were found in two patients. Classifi- (60%), respectively). Partial treatment responses were achieved cation into subgroups based on ingestion or not of daily Vita- in two immunocompromised patients (13%) and 14 immuno- min D supplements revealed mean values of 74Æ8 nmol L–1 (SD competent patients (23%). There was no significant difference =23Æ7) and 55Æ8 nmol L–1 (SD = 21Æ2) respectively. Subdivi- in the rate of adverse events (localised blistering, eczema and ur- sion of patients into daily sunavoiders and nonavoiders revealed ticaria) between the immunocompromised and immunocompe- vitamin D levels of 57Æ8 nmol L–1 (SD = 19Æ5) and 82Æ7 tent groups. We found DCP immunotherapy to be an effective nmol L–1 (SD = 28Æ3). Patients with photosensitive lupus and safe treatment for recalcitrant palmoplantar HPV infection erythematosus are adept in avoiding sunlight and are compliant in both immunocompromised and immunocompetent patients. about sunscreen use. Significantly low vitamin D levels were Our experience suggests that a longer duration of treatment is recorded in our patient cohort, 4% had critically low levels, required to achieve complete resolution in immunocomprom- below normal levels were recorded in 60% and a total of 64% ised patients. We believe that DCP immunotherapy is a valuable had levels below the optimal 80 nmol L–1 recommended by adjunct to conventional therapies for the management of cuta- many authorities. As photoprotection is necessary for disease neous HPV infection. control and as low levels also correlated with lack of oral vita- min D supplementation, we propose that patients with photo- sensitive lupus erythematosus should supplement their diet with vitamin D3. O-9 Outcome for post-transplant melanoma: a multi- centre European study R.N. Matin, C.M. Proby, J.M. McGregor, O-8 J.N. Bouwes-Bavinck,* V. del Marmol, Recalcitrant viral warts treated by diphencyprone S. Euvrard, C. Ferrandiz,§ A. Geusau,– W.L. Ho,** immunotherapy: a 5-year retrospective analysis G. Hofbauer, B. Imko, A. Lally, J. Lear,§§ comparing immunocompromised and immunocom- C. Lebbe, –– G.M. Murphy,** D. Seckin,*** petent patients E. Stockfleth, C. Ulrich, A-L. Chew, R. Morris-Jones and E. Higgins F.T. Wojnarowska and C.A. Harwood Kings College Hospital, London, U.K. Centre for Cutaneous Research, Institute of Cell and Molecular Science, Lon- Treatment of recalcitrant human papilloma virus (HPV) warts is don, U.K.; *Department of Dermatology, Leiden University Medical Center, a challenging therapeutic problem for dermatologists, especially Leiden, Netherlands; Service de Dermatologie, Hopital Erasme, Bruxelles, Bel- in immunocompromised patients. Topical immunotherapy with gium; Department of Dermatology, Edouard Herriot Hospital, Lyon, France; the contact sensitizer diphencyprone (DCP) is an effective and §Department of Dermatology, Hospital Universitario Germans Trias i Pujol, noninvasive treatment for recalcitrant HPV infection, but it is Barcelona, Spain; –Department of Dermatology, Medical University of Vienna, not widely available. Increasing numbers of transplant patients Vienna, Austria; **Department of Dermatology, Beaumont Hospital, Dublin, who require lifelong immunosuppression experience chronic Ireland; Department of Dermatology, University Hospital Zurich, Zurich, viral infections with a subsequently higher risk of trans- Germany; Department of Dermatology, Oxford Radcliffe Hospitals, Oxford, formation to cutaneous neoplasms. This study is a retrospective U.K.; §§Department of Dermatology, Manchester Royal Infirmary, Manches- analysis of our experience over a 5-year period (2000–2006) of ter, U.K.; –– Service de Dermatologie, Hopital Saint-Louis, Paris, France; treating palmoplantar HPV infections. We compared dosage, ***Department of Dermatology, Baskent University Faculty of Medicine, duration of treatment, adverse events and outcomes between Ankara, Turkey and Klinik fur Dermatologie, Venerologie und Allergolo- immunocompromised and immunocompetent patients. Sev- gie, Charite, Humbolt-Universitat, Berlin, Germany enty-six patients with recalcitrant palmoplantar viral warts were Melanoma incidence is rising faster than for any other major treated during the study period: 15 immunocompromised cancer, with an excess risk of 3 in normal subjects, and

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 Main Plenary Sessions 5

10-fold in transplant recipients. We have co-ordinated a multi- developed symptoms of EPP in adulthood, subsequently centred European study within the SCOPE network (Skin Care shown to be due to a new mutation in the FECH gene. A 37- in Organ Transplant Patients, Europe) to determine the out- year-old woman developed acute onset, painful, red, swelling comes of melanoma in transplant recipients. Retrospective data of her light-exposed skin within 24 hours of arrival in (1962–2006) were collected from 12 centres with dedicated Jamaica. She was treated with analgesics, antihistamines and transplant skin clinics across Europe and comprised 74 post- oral steroids. Since the age of 31 years she had suffered sim- transplant patients with melanoma (22 females, 52 males) and ilar, but milder, symptoms over the dorsa of her hands and six pre-transplant patients with melanoma (five females, one extensor surfaces of her forearms within hours of sun expos- male). In the post-transplant melanoma group the median age ure in the spring and summer in the U.K. Attacks settled spon- at diagnosis was 54 years (range 26–78 years) and mean taneously after two days. Her 27-year-old sister had interval from transplantation to diagnosis was 9 years (range experienced similar symptoms since the age of 18 years. Both 2–23 years) with a mean follow-up of 5 years (range 0Æ1–19 sisters were otherwise well and on no systemic medication. years). Breslow thickness was available in 58 cases, of which There was no history of consanguinity, or of other family eight (14%) were melanoma in situ, 31 (53%) were <1 mm, members being affected. When examined neither sister had nine (16%) were between 1Æ01–2 mm, four (7%) were any abnormality of their skin. On investigation, both sisters between 2.01–4 mm and six (10%) were greater than 4.0 demonstrated a plasma porphyrin spectroscopy peak at 631 mm. Post transplant melanoma survival at 5 years was similar nm and raised total erythrocyte porphyrins of 15Æ9 lmol L–1 to that published for the general population. Overall mortality and 14Æ5 lmol L–1 (normal 0Æ4–1Æ7 lmol L–1), diagnostic of from melanoma was 11 of 67 (16%) patients. The mean EPP. Full blood counts and liver biochemistry were normal interval between transplant and melanoma was 9 years (range and antinuclear and extractable nuclear antigen antibodies 1–22 years) and mean time to death was 22 months (range were negative. Analysis of both sisters’ DNA showed a new 3–67 months). In one case presentation was with metastatic mutation in exon 4 (c.418 fi A G140R) in one copy of the melanoma (primary unknown). In the remaining eight cases FECH gene and a common polymorphism in the second copy, where Breslow thickness was known, this was >4 mm in four with thymine substituted for cytosine at IVS3-48. This com- (50%), 2Æ01–4 mm in one (12%), 1Æ01–2 mm in one (12%) bination accounts for 97% of EPP in the U.K. In the recent and <1 mm in two (25%), 0Æ4 and 0Æ34 mm respectively. Six survey of EPP in the U.K. by Holme et al., 223 patients with further were present in other patients prior to EPP were investigated. The mean age of onset of their symp- transplantation, with mean age at diagnosis of 45 years (range toms was 1 year, with a range of 0–12 years. (Holme SA, An- 25–64 years) and mean interval between diagnosis and trans- stey AV, Finlay AY et al. Erythropoietic protoporphyria in the plantation of 11 years (range 0Æ4–32 years). There were no U.K.: clinical features and effect on quality of life. Br J Dermatol deaths in this group at a mean follow up of 16 years (range 2006;155:574–81). Adult onset EPP is very rare and can be 3–42 years). One patient developed a second primary mela- associated with patients undergoing leukaemic transformation. noma at 3Æ1 years post transplant and has since remained dis- This is the first family where a new germline mutation in the ease free for 6Æ3 years. Our data indicate that the overall FECH gene has been identified, which may predict adult onset outcome from melanoma post-transplant does not differ sig- EPP. nificantly from the general population despite reports from smaller series that melanomas may behave more aggressively. In addition outcome does not appear to be adversely affected O-11 in patients transplanted post-melanoma. Consistency of adalimumab response across subgroups of patients with moderate to severe psoriasis: results of the REVEAL study O-10 A. Menter, K. Papp,* C. Leonardi, R.G. Langley New mutation identified in adult onset erythropoietic and M. McIlraith§ protoporphyria Texas Dermatology Research Institute, Dallas, TX, U.S.A.; *Probity Medical J. Azad, S. Whatley,* M. Badminton* and Research, Waterloo, ON, Canada; Central Dermatology Inc., St. Louis, A.J. Carmichael MO, U.S.A.; Dalhousie University, Halfax, NS, Canada and §Abbott Department of Dermatology, James Cook University Hospital, Middlesbrough, Laboratories, Ludwigshafen, Germany U.K. and *Department of Medical Biochemistry and Immunology, Cardiff This analysis was performed to determine whether specific University, Cardiff, U.K. characteristics of patients or their disease altered the efficacy Erythropoietic protoporphyria (EPP) is a disorder of haem bio- of fully human monoclonal antibody, adalimumab, in the synthesis. It normally presents in childhood and results from a REVEAL clinical study. Patients with moderate to severe (PASI deficiency of the ferrochelatase enzyme, which is coded for by ‡ 12) psoriasis who had failed topical therapy and had not the FECH gene. A number of mutations in the FECH gene have previously had anti-TNF therapy were enrolled in REVEAL, a been described. To express EPP, individuals usually have a 52-week study of adalimumab. In the first 16-week double- mutation in one of their FECH genes and a low expression blind, placebo-controlled period (Period A), patients were variant on the other allele. We describe two sisters who only randomized to receive either placebo or adalimumab (80 mg

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 6 Main Plenary Sessions at Week 0 then 40 mg every other week from Weeks 1–15). treated with NB-UVB thrice weekly. The initial dose was 70% The primary analysis was the percentage of patients achieving of minimal dose (MED); thereafter 20% increments ‡PASI 75 at Week 16. Subgroup analyses were conducted to were given at each visit. Patients were treated with PUVA explore the effect on treatment outcomes of age, duration of three times or twice weekly based on skin type (before 1998) psoriasis, medical history of psoriatic arthritis, and recent or minimal phototoxic dose (MPD) (after 1998). Incremental (within the past 12 months) systemic biologic (except anti- increases were given once weekly based on skin type (before TNF) or non biologic therapy. Overall, 1,212 patients at 81 1998) or at each visit based on 20% of UVA dose (after sites in the U.S.A. and Canada were randomized 2 : 1 to 1998). The patient’s first course of treatment was used for receive adalimumab (814) or placebo (398). At Week 16, comparison. Patients were treated until the skin was clear and 71% of patients treated with adalimumab (mean age 44 years) maintenance therapy was not given. A complete response achieved ‡PASI 75, vs. 6% of patients treated with placebo (P (CR) was defined as less than 1% extent, a partial response <0Æ001). Subgroup analyses were performed on those (PR) as >50% improvement and a failed response (FR) as patients <40, 40–64, and (65 years of age. The mean dura- <50% improvement. Relapse was defined as clinically signifi- tion of psoriasis was 18 years. Subgroup analyses were per- cant disease requiring further therapy and a relapse-free inter- formed by quartiles of duration of psoriasis. Of the patients val as the period between CR and relapse. Forty patients, 26 treated with adalimumab, 224 had a medical history of psoria- male and 14 female, with a mean age of 70 years (range 28– tic arthritis, 97 and 188 had received recent systemic biologic 82) were included in the study. The clinical stage at the time or nonbiologic therapy, respectively. The efficacy of adalimu- of treatment was: IA (NB-UVB 6, PUVA 7), IB (NB-UVB 4, mab treatment was similar across all subgroups of patients PUVA 14), IIA (NB-UVB 1, PUVA 3), IIB (NB-UVB 1, PUVA (Table 1). Adalimumab was well tolerated, with 96% of 2), III (PUVA 1) and IVA (PUVA 1). Twelve patients had NB- patients completing period A vs. 89% for placebo. The per- UVB treatment with CR in six patients, PR in four, and FR in centage of patients experiencing serious infectious adverse two. The patients had a median of 19 treatments (range 8– events were 0Æ6% for adalimumab vs. 1.0% for pla- 53) with a median cumulative dose of 12Æ6Jcm-2 (range cebo. Results from the first 16-week period of this 52-week 3Æ5–105Æ6). Twenty-eight patients were treated with PUVA study suggest that adalimumab was efficacious regardless of therapy with CR in 18 patients, PR in six, and FR in four. The age, duration of psoriasis, diagnosis of psoriatic arthritis, or patients had a median of 19Æ5 treatments (range 7–50) with a ) recent systemic therapy, including biologic therapy. median cumulative UVA dose of 31Æ7Jcm 2 (range 10–215). The median relapse-free interval for patients treated with Table 1. Psoriasis Area and Severity Index 75 responses in patients PUVA was 10.5 months (range 1–33) and for patients treated treated with adalimumab with NB-UVB was 7 months (range 1–23). A Mann–Whitney

Recent test showed no statistically significant difference between the History systemic number of exposures for each group (P =0Æ7). There was no Age (y) Quartiles of disease duration (y) of treatment psoriatic statistically significant difference between the relapse-free <40 40–64 >65 0Æ5–9Æ79Æ7–15Æ915Æ9–24Æ824Æ8–65 arthritis biol nonbiol intervals for NB-UVB and PUVA (P = 1). Adverse effects % PASI 75 74 70 61 68 67 76 73 70 78 68 included grade 2 erythema in 12 patients (NB-UVB = 7, PUVA 5), nausea and pruritus in two patients (PUVA). Clear- All results, P <0Æ001 vs placebo in each subgroup. ance rates and relapse free interval were similar in both groups for those with early stage disease. Conflict of interest: The REVEAL study was sponsored by Abbott Laboratories and M.McI. is an employee of Abbot Laboratories. O-13 Keratinocytes from patients with polymorphic light eruption differentially express genes related to clearance of apoptotic cells O-12 R.A. Palmer, K. Kaneko,* A.R. Young,* Narrowband ultraviolet (UV) B and psoralen UVA J.M. McGregor, J.L.M. Hawk* and S.L. Walker* therapy in the treatment of : a King Edward VII Hospital, Windsor, U.K.; *St. John’s Institute of Derma- retrospective study tology, London, U.K. and Centre for Cutaneous Research, London, U.K. K. Ahmad, S. Rogers, P. McNicholas and Polymorphic light eruption (PLE) is the result of an aberrant P. Collins immunological reaction of the skin to ultraviolet radiation St Vincent’s University Hospital, Dublin, Ireland (UVR). In this study we employ genome-wide expression an- This retrospective study was carried out to analyse the alysis to identify genes involved in the response to UVR that response to treatment, relapse-free interval and adverse effects are differentially expressed between patients with PLE and of narrowband ultraviolet (UV)B (NB-UVB) phototherapy and controls. Keratinocyte cultures from eight patients with severe psoralen (P)UVA in patients with histologically confirmed my- PLE and eight control subjects, 24 hours after becoming 100% cosis fungoides (MF) between 1980 and 2003. Patients were confluent, were sham irradiated or exposed to solar-simulated

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 Main Plenary Sessions 7 radiation (SSR) at a dose of 970 mJ cm–2 (measured as the accurately in the patient’s records in 17 (59%) and interacting UVB component of SSR). Six hours later RNA was extracted sensitively with people with skin disease in 25 (86%). Import- and the expression levels of over 22 000 genes were analysed ant Outcomes: we found wide variation in what is included in using Affymetrix HG-U133A 2Æ0 arrays and Genespring curricula. Core dermatology teaching mostly takes place in the vÆ7Æ3Æ1 software. By analysis of variance, 1979 genes were 4th year (59%). In all medical schools teaching takes place in found to be regulated by UVR in both PLE and controls. The secondary care and in 16 (55%) of the medical schools teach- gene most upregulated in both groups was cyclooxygenase-2. ing also takes place in primary care. Dermatologists are re- However, expression levels of just 16 genes were differentially sponsible for teaching the undergraduates in all medical expressed in PLE and control keratinocytes. For eight of these schools, but Specialist Nurses also have a role in 20 (69%) of we attempted to confirm the array results with real-time poly- the medical schools and GPs in 10 (34%) of the medical merase chain reaction (PCR), and we succeeded for six genes. schools. Expert patients are involved in teaching in 6 (21%) Of the 16 genes, six may be involved in the clearance of of the medical schools. In 28 (97%) of the medical schools apoptotic cells, namely complement 1 s subunit, scavenger re- there are opportunities for extra dermatology experience such ceptor B1, fibronectin-1, immunoglobulin superfamily mem- as Special Study Modules. All medical schools use learning and ber-3, caspase-1 and paraoxonase-2. This novel finding, that teaching during outpatient clinics with lectures being used in of the few genes differentially expressed in PLE, many have 28 (97%) medical schools and small group teaching in 27 functions associated with the clearance of apoptotic cells (93%). Observation of clinical nurse specialists occurs in only implicates this process in the pathogenesis of PLE. It is intrigu- 14 (48%) of medical schools. Knowledge based assessments ing that defective clearance of apoptotic cells is also believed such as MCQs and EMQs are used in 28 medical schools to play a pathogenic role in lupus erythematosus (Savill J, (97%) and OSCEs are in 21 (72%). This audit has established Dransfield I, Gregory C, Haslett C. A blast from the past: clear- areas of good practice and identified areas where changes are ance of apoptotic cells regulates immune responses. Nat Rev needed in the dermatology undergraduate curricula of medical Immunol 2002; 2: 965–75), and many patients with lupus schools. These comparative data will provide a strong case for erythematosus also have PLE (Millard TP, Lewis CM, Kha- developing the learning and teaching of dermatology in areas mashta MA et al. Familial clustering of polymorphic light erup- where they are currently lacking. Project supported by the tion in relatives of patients with lupus erythematosus: British Skin Foundation. evidence of a shared pathogenesis. Br J Dermatol 2001; 144: 334–8). We postulate that PLE results from aberrant clearance of apoptotic cells from the epidermis following exposure of the skin to UVR. O-15 A homozygous missense mutation in TGM5 causes O-14 acral peeling skin syndrome in two English brothers Audit of dermatological content of U.K. A. Cassidy, C. Moss,* A. Wong * and W.H.I. McLean undergraduate medical curricula in 2006 University of Dundee, Dundee, U.K. and *Birmingham Children’s Hospital, E. Davies and S. Burge Birmingham, U.K. Department of Dermatology, Oxford Churchill Hospital, Oxford, U.K. The peeling skin syndromes are genetic disorders characterized Recommendations for the core undergraduate curriculum for by shedding of the outer epidermis. The acral form can be dermatology were circulated to all medical schools in June confused with epidermolysis bullosa simplex but true 2006 (Dermatology in the Undergraduate Medical Curriculum. do not occur. The genetic cause of acral peeling skin syn- Recommendations of the British Association of Dermatologists, drome (APSS) has recently been identified as TGM5 which June 2006). These were informed by the results of a modified encodes the epidermal enzyme transglutaminase 5 (Cassidy AJ, Delphi study, which identified the most important learning van Steensel MA. Steijlen PM et al. A homozygous missense outcomes in dermatology (Clayton R, Perera R, Burge S. Defi- mutation in TGM5 abolishes transglutaminase 5 activity and ning the dermatological content of the undergraduate medical causes acral peeling skin syndrome. Am J Hum Genet 2005;77: curriculum: a modified Delphi study. Br J Dermatol 2006; 155: 909–17). We report the first English family with APSS due to 137–44). We designed a questionnaire to audit the core curri- TGM5 mutation. Two brothers, now aged 2 and 5 years, culum in each U.K. medical school against the recommended developed peeling of the digits from a few weeks of age. Epi- standards in the new curriculum. This was piloted and then sodes of peeling occurred every two or three weeks through- sent to one Irish and all U.K. medical schools. We received 29 out the year with no obvious precipitants. There was no replies (97% of 30). Essential Clinical Outcomes: taking a family history of skin peeling or of consanguinity. On exam- dermatology history is part of the curriculum in 25 (86%) ination the palms and soles were pink and shiny with several medical schools, exploring a patient’s concerns and expect- areas of superficial peeling; the abnormal skin extending ations in 24 (83%), examining skin, hair and nails in 25 around the nails distally. DNA was extracted from blood for (86%), examining mucous membranes in 21 (72%), descri- mutation analysis of the TGM5 gene, which consists of 13 ex- bing cutaneous physical signs in 25 (86%), recording findings ons spanning 33Æ7 kb of genomic DNA, encoding two major

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 8 Main Plenary Sessions splice variants. Mutation analysis of all exons and splice sites alternatively they may provide help for B-cell generation of revealed two homozygous missense changes, T109M and antibodies against the BMZ autaoantigens. This is the first G113C. The same two homozygous mutations occurred in the demonstration of a specific T-cell response in LS. Overall our Dutch kindreds reported previously (Cassidy et al.) In that data suggests that LS is characterized by an autoimmune, pre- study a biochemical assay revealed the pathogenicity of the dominately T-cell response to a structural protein, BP180, in G113C variant and that the T109M variant did not affect the basement membrane zone of the skin. The basement expression and probably represents a rare polymorphism. membrane zone autoantibodies may have a transient or ampli- Three-dimensional modelling of transglutaminase 5 showed fying role in the pathogenesis of LS. These findings are the that G113C lies close to the catalytic domain and would prob- first to show the presence of circulating basement membrane ably disrupt the protein cross-linking function performed by zone autoantibodies and a T-cell response to a basement mem- this enzyme that appears to be vital for maintaining cell-cell brane zone antigen in the same patients with vulval LS. They adhesion between the outermost layers of the epidermis. APSS suggest that autoimmune mechanisms, both T-cell and autoan- is easily mistaken for epidermolysis bullosa simplex, Weber- tibody responses, to the basement membrane zone may con- Cockayne type (EBS-WC), because patients often report tribute significantly to the pathogenesis of . ‘blisters’, however, the former is recessively inherited and the latter dominant. TGM5 is another candidate gene to consider in sporadic patients diagnosed as EBS-WC who have no mutation in the KRT5 and KRT14 genes. This family clearly O-17 demonstrates the classical phenotype of APSS. A review of 241 subjects patch-tested twice J. White, J.P. McFadden and I.R. White Department of Cutaneous Allergy, St. John’s Institute of Dermatology, London, U.K. O-16 Conflicting data exists regarding active sensitization from Vulval lichen sclerosus is associated with an patch testing. This has led to the withdrawal of PPD from the autoantibody and T-cell response to basement standard series in Germany. Subjects who have been patch tes- membrane zone antigens ted more than once may shed light on the phenomenon of M. Baldo, B. Bhogal,* A. Bailey, G. Ogg and active sensitization, as well as cases where patch test findings F.T. Wojnarowska are not reproduced. Methods: Patients patch tested more than University of Oxford, Oxford, U.K. and *St John’s Institute of Dermatology, once in our department were identified from our computer- London, U.K. ized database and case notes by their unique unit number, Lichen sclerosus (LS) is a chronic inflammatory skin disease name and date of birth. Patients undergoing repeat patch tests associated recently with the demonstration of circulating base- within 30 days were excluded from the study. Statistics were ment membrane zone autoantibodies in 30% of patients, undertaken using a web v2 calculator. Results: Two hundred which provides direct evidence of an autoimmune process. and forty-one subjects who had been patch tested twice were However, LS is characterized histologically by an inflammatory identified, having ages with a Gaussian distribution. The mean T-cell infiltrate, and it seems more likely that LS is mediated age difference between the two tests was 5 y (median 5 y). by lymphocytes. The aim of the present study was to demon- Headline positivity was similar for the following allergens: strate the presence of circulating basement membrane zone nickel; myroxylon pereirae; para-phenylenediamine (PPD); autoantibodies and an associated T-cell response in the same colophonium; epoxy resin; neomycin; quaternium-15; thiu- vulval LS patients. We recruited 16 patients with vulval LS and ram mix; sesquiterpene lactone and p-tert-butylphenol formal- four healthy controls. Indirect immunofluorescence for detec- dehyde (PTBP) resin. Headline positivity was statistically tion of IgG and IgA basement membrane zone antibodies in significantly higher for fragrance mix I on the second test the patient’s sera was undertaken using a standard technique. compared with the first (29 of 241 or 12% for the first test In addition we isolated peripheral blood mononuclear cells and 39 of 241 or 16% for the second test). Analysing the data (PBMC) from the same patients and controls and cultured from those patients repeatedly patch tested within 12 months them in vitro. The cultured PBMC were examined for their im- (who had a similar age profile to the whole group), the munogenicity for overlapping peptides spanning the NC16A results were similar to the data above: only fragrance mix I domain using interferon (IFN)-gamma enzyme-linked immu- showed a statistically significant increase at the second test (1 nospot assay (Elispot). Indirect immunofluorescence showed of 42 or 2% for the first test and 5 of 42 or 12% for the sec- linear staining at the basement membrane zone in four ond test). Expected rates of positivity for fragrance mix I were patients (25%), we observed a significant IFN-c production in calculated from previously published data from our group, response to the NC16A peptides in five patients (31%), but according to age and gender (Buckley DA, Rycroft RJG, White not in any of the normal controls. Three of the five patients IR, McFadden JP. The frequency of fragrance allergy in patch- (60%) who had positive Elispot responses also had BMZ auto- tested patients increases with their age. Br J Dermatol 2003; 149: antibodies. However, B lymphocytes (helper) produce autoan- 986-9). Again this shows a much larger than expected number tibodies to the BMZ and these T cells may be effector cells, of positives (age and sex controlled) at the second testing,

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 Main Plenary Sessions 9 compared with the first. Discussion: This study’s findings are O-19 particularly important since the testing was undertaken in the Adalimumab treatment is associated with more rapid same department by the same observers, hence reducing inter- improvement in psoriasis versus methotrexate and observer variation. There is a statistically significant increased versus placebo: results from the CHAMPION study risk of positivity on the second test compared with the first, J-H. Saurat, R. Langley,* G. Stingl and M. McIlraith similarly in those who were tested twice within 12 months. Hospital Cantonal Universitaire, Geneva, Switzerland; *Dalhousie University, Age is not relevant in this subgroup and usage of fragrance Halifax, Canada; Universitaetsklinik Fuer Dermatologie, Vienna, Austria products is likely to remain constant. This excludes two com- and Abbott Laboratories, Ludwigshafen, Germany mon confounding factors. Explanations for the significant dif- This analysis was performed to compare time to PASI response ference would include a number of possibilities, including for adalimumab, a fully human anti-TNF monoclonal antibody, active sensitization, gross changes in usage or exposure vs. methotrexate and vs. placebo in a Phase III trial, CHAMPION (despite subgroup analysis) and seasonal factors. It would be (comparative study of adalimumab vs. methotrexate and vs. pla- imprudent to draw too many conclusions from this small cebo in psoriasis patients), in patients with moderate to severe study. Results from other centres may help to confirm or chronic plaque psoriasis. Patients with moderate to severe psor- refute this striking observation. iasis, who enrolled in CHAMPION, a 16-week randomized, double dummy trial, were randomized to one of three treatment arms: (i) adalimumab 80 mg subcutaneously at Week 0 and 40 O-18 mg every other week from Weeks 1–15, (ii) oral methotrexate, Comparison of effectiveness and safety of azathioprine versus betamethasone for the starting at 7.5 mg per week and advancing as tolerated and indi- treatment of Parthenium dermatitis cated to a maximum dose of 25 mg per week, or (iii) placebo. K. Verma, R. Mahesh, P. Srivastava and M. Ramam The primary endpoint was the proportion of patients with ‡PASI All India Institute of Medical Sciences, New Delhi, India 75 response at Week 16. Times to ‡PASI 50 and 75 responses Parthenium dermatitis is a challenging problem to treat. Cortico- were also assessed. Intention-to-treat analyses were performed steroids are the mainstay of treatment, however, they may on all groups. Missing data were considered a nonresponse for cause serious side effects after prolonged use. Azathioprine has the primary endpoint and were censored at last observation for been shown to be effective. We evaluated the effectiveness time-to-event analyses. A total of 271 patients enrolled in and side effects of azathioprine versus betamethasone in this CHAMPION. Mean PASI scores at baseline were 20Æ2, 19Æ4, and disease. The study was funded by the Council of Medical 19Æ2 for the adalimumab, methotrexate, and placebo groups, re- Research. Fifty-five patients with positive patch tests for Parthe- spectively. Of patients treated with adalimumab, 80% reached a nium dermatitis were randomized into two groups. Group A ‡PASI 75 response at Week 16 vs. 36% of methotrexate patients patients were treated with azathioprine 50 mg twice daily and (P <0Æ001) and vs. 19% of placebo patients (P <0Æ001). The group B patients with betamethasone 1 mg twice daily for 6 median time to a ‡PASI 50 response was 28 days in adalimu- months in a blinded manner. Response to treatment and side mab-treated patients compared with 84 days in methotrexate- effects were evaluated every month. The patients were then treated patients. The median time to a ‡PASI 75 response was followed-up for another 6 months to determine the relapse 56 days in adalimumab-treated patients compared with 113 rate. Forty-one patients, 20 in group A and 21 in group B, days in methotrexate-treated patients. The median time to PASI completed the study. Six patients in group A and eight in 50 and PASI 75 could not be calculated for placebo patients group B were lost to follow-up for various reasons. Nineteen because the Kaplan–Meier estimate at the end of the observation patients in group A and all 21 patients in Group B had an period was still above 50% for the placebo treatment group. The excellent response (complete remission) to treatment. The times from baseline to ‡PASI 50 and ‡PASI 75 responses were mean clinical severity score (CSS) decreased from 64Æ5±16Æ4 statistically significantly shorter with adalimumab treatment to 4Æ3±5Æ6(P =0Æ015) in group A while it decreased from compared with methotrexate and with placebo treatment (P < 67Æ1±17Æ4to0Æ6±2Æ2(P =0Æ0005) in group B. A statistic- 0Æ001). Adalimumab treatment was associated not only with a ally significant decrease in CSS was shown in both the groups statistically significant superior proportion of patients achieving (Fisher’s exact test, P =0Æ04). There was no statistically signi- improvement at Week 16, but also with a statistically signifi- ficant difference in response rate between the two groups. The cantly more rapid improvement, vs. methotrexate and vs. pla- side effects were more frequently seen in group B. Nine cebo, in this Phase III psoriasis trial. This was the first study to patients in group A and 14 in group B relapsed during the demonstrate that a biological agent compared with a systemic post-treatment follow-up. This study has shown that azathiop- non-biologic agent was associated with more rapid improve- rine and betamethasone are equally effective for the treatment ments in psoriasis. Conflict of interest: The CHAMPION study was sponsored by of Parthenium dermatitis. However, the side effects and relapses Abbott Laboratories, and MMcI is an employee of Abbott are more frequent in patients treated with betamethasone. Laboratories.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1): 1–9 Registrars’ Symposium

RF-1 human skin substrates (32% and 30%, respectively) and in Pulsed intravenous cyclophosphamide and antidesmoglein 1 and 3 autoantibody levels (40% and 21%, methylprednisolone therapy is effective in respectively) from pre- to post-last pulse. We conclude that refractory pulsed intravenous therapy with cyclophosphamide and methyl- M. Saha, A.-M. Powell, B. Bhogal, M.M. Black and prednisolone appears to be an effective and safe treatment for R.W. Groves refractory cases of pemphigus. However, the optimum dosing St John’s Institute of Dermatology, London, U.K. frequency and duration remain uncertain and further con- Pemphigus is a rare autoimmune blistering disorder. The trolled studies are required in order to define the optimal mainstay of current pemphigus treatment is high-dose oral treatment regimen. corticosteroid therapy in combination with steroid-sparing agents. However, some patients do not respond to this regime and pulsed therapy with intravenous cyclophosphamide and methylprednisolone has been shown to be an effective alter- native treatment and may be less toxic than daily oral therapy. RF-2 However, there are currently few large-scale studies of this The quality of life of 797 patients with approach. This study represents a retrospective review of photodermatoses patients treated with pulsed methylprednisolone and cyclo- C.T. Jong, A.Y. Finlay, A.V. Anstey, A.D. Pearse, phosphamide (PPC) therapy in our unit over the past 10 A.C. Kerr,* J. Ferguson,* E.C. Benton, J.L.M. Hawk, years. We have reviewed the clinical and immunological R.P. Sarkany, E. McMullen, L.E. Rhodes and responses of 21 patients with refractory severe pemphigus P.M. Farr§ treated with a standardized protocol of monthly PPC. Patients Department of Dermatology, Cardiff University Wales College of Medicine, received intravenous methylprednisolone 1 g daily for 3 days Cardiff, U.K.; *Photobiology Unit, Ninewells Hospital and Medical School, with cyclophosphamide 500 mg on the second day, repeated Dundee, U.K.; Photodermatology Unit, St John’s Institute of Dermatology, at monthly intervals. The oral prednisolone dose was titrated St Thomas’ Hospital, London, U.K. Photobiology Unit, Dermatology Centre, according to clinical response and most patients also received Hope Hospital, Manchester, U.K. and §Department of Dermatology, Royal cyclophosphamide 50 mg daily. All patients in the study had Victoria Infirmary, Newcastle, U.K. previously failed to respond to high-dose prednisolone and Photodermatoses may have a major impact on patients’ multiple adjuvant immunosuppressants such as azathioprine quality of life (QoL). The aim of this study was to assess or mycophenolate mofetil. There was a demonstrated the impact of all forms of photodermatoses on patients’ improvement in oral and skin disease activity scores from QoL. From five U.K. centres, 1886 patients who had been pre- to post-last pulse values in all patients. Results showed seen between 2000 and 2005 were contacted and asked to that of the 21 patients treated, eight were assessed as having complete Dermatology Life Quality Index (DLQI) question- an excellent (> 90% clinical improvement) response, eight a naires, sent out on 7 June 2006 during a forecasted and good (30–90% improvement) response and five a minimal actual sunny week across the U.K. Overall, 797 (43%) (< 30% improvement) response. The average number of patients replied, with a range of 31–48% for the individual treatment pulses given was 11 (range 4–22). Four patients centres. The results of the study are as shown in the table achieved complete clinical remission with follow-up periods below. This is the first large-scale study to attempt to meas- of between 3 and 6 years. The number of pulses for these ure the impact of photodermatoses on QoL. Nearly two out patients varied between a minimum of 11 and a maximum of of three patients with actinic prurigo (AP), and more than 22. Change in daily prednisolone dose may be regarded as a one out of three patients with photoaggravated dermatoses good surrogate indicator of clinical change in disease activity (PAD), chronic actinic dermatitis (CAD), erythropoietic pro- and we found that all patients were able to reduce their daily toporphyria (EPP) and solar urticaria (SU) had a DLQI of prednisolone dose (pre-pulsing mean average dose 40 mg, > 10 confirming a very large effect of the disorders on last pulse mean 114 mg, with an average mean dose reduc- QoL. The median scores of AP and PAD are comparable to tion of 65%). Adverse effects of treatment were minimal; the that of severe atopic dermatitis and severe psoriasis. Both most common adverse effect was transient lymphopenia variegate porphyria (VP) and (PCT) (57%). Seven episodes of nonfatal sepsis (33%) were recor- had a much lower impact on QoL than EPP. Only two ded during PPC therapy. All patients demonstrated improve- patients with xeroderma pigmentosum (XP) replied, and the ment in oral and skin scores from pre- to post-last pulse unusually low impact on QoL may reflect a mild pheno- values. This was matched by a reduction in indirect immuno- type. AP has the highest score, presumably reflecting its fluorescence titres using both monkey oesophagus and normal persistence and poor response to treatment.

2007 The Authors 10 Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 10–13 Registrars’ Symposium 11

weeks. One patient with excellent response reported depres- Number of Mean Mean disease Median % of patients sion which settled on stopping the drug at week 64. A 46- patients age duration DLQI with Disease (female) (years) (years) score DLQI > 10 year-old man died suddenly at week 36 from what is thought to be unrelated causes. Etanercept is an effective and well-tol- AP 26 (23) 45 23 13 62 PAD 114 (81) 53 15 10 48 erated treatment for severe psoriasis. Development of tubercu- CAD 128 (60) 63 19 9 39 losis in one patient underlines the need for rigorous EPP 19 (13) 44 40 8 37 tuberculosis screening. Our results also suggest that etanercept SU 50 (33) 48 14 9 36 can be used intermittently without rebound and that psoriasis PLE 386 (331) 45 14 8 27 responds again on reintroduction of etanercept. PCT 12 (4) 57 13 1.5 25 DIP 28 (20) 63 11 4 21 PCD 20 (9) 54 11 2 20 VP 3 (2) 45 17 3 0 XP 2 (2) 42 20 3 0 RF-4 PLE, polymorphic light eruption; DIP, drug-induced photoder- Fibroblast activation by magnetic resonance imaging matoses; PCD, photocontact dermatitis. contrast agent: a cause of nephrogenic fibrosing dermopathy? S. Bhattacharya, J. Quinn, A. Burden and M. Edward Department of Dermatology, Western Infirmary, Glasgow, U.K. Nephrogenic fibrosing dermopathy (NFD), also known as RF-3 nephrogenic systemic fibrosis was first documented in 1997 Two years’ experience with etanercept in recalcitrant and to date its pathogenesis remains unknown. It is confined psoriasis to patients with renal disease and presents as grossly thickened K. Ahmad and S. Rogers tight hard skin that feels woody. Histology reveals a dermis St Vincent’s University Hospital, Dublin, Ireland containing thickened collagen bundles with surrounding clefts, This retrospective study was carried out to assess the efficacy a variable amount of mucin and large plump fibroblast-like and safety profile of etanercept in 49 patients with recalcitrant cells. There has been a recent suggestion of a link between the psoriasis over 2 years. Written consent was obtained. Pretreat- use of a particular magnetic resonance imaging (MRI) contrast ment screening included full blood count, urea and electro- agent, gadalinium chelate and the onset of this disease. The lytes, lung function test, antinuclear antibodies and screening aim of this study is to determine levels of NFD fibroblast hya- for tuberculosis with the Mantoux test and a chest X-ray. luronan synthesis and assess the effect of this particular MRI Response to treatment was assessed by Psoriasis Area and contrast agent on fibroblasts. We established fibroblasts from Severity Index (PASI) score and Physician Global Assessment the fibrotic lesions of six patients with NFD, and as the strong (PGA). Patients were reviewed at 8-week intervals when clin- Alcian blue staining suggested elevated levels of hyaluronan, ical response and adverse effects were noted. All patients had we compared hyaluronan synthesis by the NFD fibroblasts to failed to respond to, or were intolerant of, treatments includ- that of two normal fibroblast cultures using an enzyme-linked ing narrow-band ultraviolet (UV) B radiation (64%), psoralen immunosorbent-type assay. Serum from the patients with NFD plus UVA (89%), methotrexate (81%), ciclosporin (61%), was also assessed for fibroblast hyaluronan-stimulating activity. acitretin (31%), fumaric acid esters (26%), hydroxyurea Gadalinium chelate was assessed for its effect on fibroblast (22%) and infliximab (14%). Thirty-nine (79%) received proliferation in monolayer and collagen lattice cultures. NFD etanercept 25 mg twice weekly; 10 patients (20%) with severe fibroblasts synthesized excess levels of hyaluronan compared disease had 50 mg twice a week. Six patients had a positive to control fibroblasts (up to 10-fold increase), while patient Mantoux and one had an abnormal chest X-ray. They were serum stimulated control fibroblast hyaluronan synthesis, and treated with isoniazid for 9 months. Forty-four patients (89%) when combined with patient fibroblasts, hyaluronan synthesis had chronic plaque psoriasis, two (4%) were suberythroder- was increased up to 19-fold compared to control cultures. mic, one had palmoplantar pustular psoriasis and two (4%) Gadalinium added to culture medium stimulated fibroblast had acrodermatitis continua of Hallopeau. PASI 75 was growth in a dose-dependent manner with cell numbers in- achieved in 47% of patients at week 24 and 66% at week 48. creasing 2-fold following 5 days of incubation with 1 mmol At week 24, 44% had a PGA score of excellent, and at week gadalinium chelate. Exposure of fibroblasts to gadalinium for 48, 58% scored excellent. In 12 who cleared, etanercept was only 3 h followed by washing and incubation in control med- stopped. Eight of these relapsed and etanercept was recom- ium stimulated growth as if the gadalinium was present con- menced while four remained in remission for a mean of 13 tinuously suggesting the cells were activated in some way by weeks (range 6–20). One patient developed injection site the gadalinium chelate. This report is the first to demonstrate reactions which Koebnerized rapidly resulting in a generalized a role for gadalinium in stimulating fibroblasts, suggesting the flare of psoriasis. A patient with negative tuberculosis screen- increased fibroblast cell numbers caused by gadalinium chelate ing, developed cervical tuberculous lymphadenitis after 24 may contribute to the observed fibrosis.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 10–13 12 Registrars’ Symposium

RF-5 RF-6 Dermatological conditions in intensive care: analysis Does the presence or absence of type VII collagen of a national intensive care audit database expression in individuals with recessive dystrophic S. George, D. Harrison,* C. Welch,* K. Nolan and epidermolysis bullosa have implications for the risk P. Friedmann of developing squamous cell carcinoma? Southampton University Hospitals NHS Trust, Southampton, U.K. and G.A. Cox, C. Pourreyron,* A. Volz, *Intensive Care National Audit & Research Centre, London, U.K. E.A. O’Toole,* M. Chen, L. Bruckner-Tuderman, Dermatology is usually thought of as an outpatient specialty J.C. Salas-Alanis,§ I.M. Leigh,* A.P. South* and with low mortality; however, some skin conditions require J.A. McGrath intensive care, e.g. toxic epidermal necrolysis (TEN), Ste- Genetic Skin Disease Group, St John’s Institute of Dermatology, St Thomas’ vens–Johnson syndrome, necrotizing fasciitis and exfoliative Hospital, London, U.K.; *Centre of Cutaneous Research, Institute of Cell and dermatitis. These conditions are rarely seen; the incidence Molecular Science, Barts and The London, London, U.K.; Department of of TEN is reported to be between 0Æ4 and 1Æ3 cases per Dermatology, University of Freiberg, Freiberg, Germany; Department of million person-years. This makes it difficult for individuals Dermatology, Keck School of Medicine, University of Southern California, to gain experience of managing such cases. Conditions with Los Angeles, CA, U.S.A. and §Servicios Medicos de la Universidad such low incidence are best studied using clinical databases Autonoma de Nuevo Leon, Monterrey, Mexico arising from multicentre audits or disease registries. We Recessive dystrophic epidermolysis bullosa (RDEB) is caused interrogated a large, high-quality clinical database from a by loss-of-function mutations on both alleles of the COL7A1 national audit of adult intensive care units (ICUs), the Case gene which result in reduced or undetectable expression of Mix Programme (CMP), with the aim of identifying and type VII collagen at the dermal–epidermal junction (DEJ). characterizing patients with dermatological conditions requi- Clinically, the degree of blistering, ulceration and scarring in ring admission to ICU. Data were extracted for 476 224 individuals with RDEB appears to correlate, to some extent, admissions to 178 ICUs participating in the CMP over the with the amount of type VII collagen that is present at the time period December 1995 to September 2006. A total of DEJ. Of note, in the most severe Hallopeau–Siemens form of 2245 dermatological admissions were identified, summarized RDEB, type VII collagen is typically absent. One of the major in Table 1. These represent 0Æ5% of all ICU admissions, or complications of RDEB is the development of squamous cell approximately 2Æ1 dermatological admissions per ICU per carcinoma (SCC) which can occur from the second decade of year. We have identified patients who not only require life and which is associated with increased mortality (over intensive care, but also dermatological care. Many of these 55% of individuals with Hallopeau–Siemens RDEB will die conditions have a high mortality and it is essential that such from SCC before the age of 40 years). Historically, clinical patients have access to a dermatologist. This highlights the experience has suggested that the risk of SCC is greatest in important role of dermatologists in an acute hospital RDEB subjects who have the most severe blistering and scar- setting. ring and, by implication, a complete absence of type VII colla- gen expression at the DEJ. However, this dogma was challenged by a recent report (Ortiz-Urda S, Garcia J, Green CL et al. Type VII collagen is required for Ras-driven human Table 1. Dermatological admissions to intensive care epidermal tumorigenesis. Science 2005; 307: 1773–6) which ICU ICU ICU Hospital found type VII collagen expression in 10 RDEB SCCs with evi- admissions, deaths, transfers, deaths, dence to suggest that presence of the noncollagenous domain n (%) n (%) n (%) n (%) (NC1) of type VII collagen was critical for tumour invasion. Necrotizing fasciitis 1133 (0Æ24) 336 (29Æ7) 56 (4Æ9) 438 (41Æ6) This infers that cases of RDEB with a complete absence of type Cutaneous 657 (0Æ14) 193 (29Æ4) 14 (2Æ1) 274 (42Æ7) VII collagen are at less risk of developing SCC compared with Orbital cellulitis 48 (0Æ01) 8 (16Æ7) 1 (2Æ1) 12 (26Æ7) those who express the NC1 domain in their skin. To evaluate Cutaneous melanoma 79 (0Æ02) 5 (6Æ3) 0 (0Æ0) 12 (15Æ8) this new concept in a different cohort of RDEB subjects, we Basal cell carcinoma 98 (0Æ02) 6 (6Æ1) 0 (0Æ0) 11 (11Æ7) assessed type VII collagen expression in 16 previously excised of skin SCCs using polyclonal antitype VII collagen antibodies for Toxic epidermal 86 (0Æ02) 32 (37Æ2) 5 (5Æ8) 43 (50Æ6) necrolysis immunofluorescence microscopy (frozen tissue sections) and Stevens–Johnson 46 (0Æ01) 14 (30Æ4) 5 (10Æ9) 19 (46Æ3) Western blotting (cultured SCC keratinocytes). Presence of syndrome type VII collagen was detected in 13 of 16 tumours but in 19 (0Æ00) 3 (15Æ8) 2 (10Æ5) 6 (31Æ6) three SCCs (from two different patients) there was a complete Exfoliative dermatitis 15 (0Æ00) 6 (40Æ0) 0 (0Æ0) 6 (40Æ0) absence of type VII collagen. One of these subjects was a com- 9 (0Æ00) 4 (44Æ4) 0 (0Æ0) 4 (44Æ4) pound heterozygote for two nonsense mutations in COL7A1 Psoriasis/pustular 19 (0Æ00) 8 (42Æ1) 0 (0Æ0) 8 (42Æ1) (p.R525X and p.R578X) both of which occur upstream of the psoriasis 36 (0Æ01) 15 (41Æ7) 1 (2Æ8) 19 (54Æ3) putative NC1 tumour invasion-promoting site proposed by Total 2245 (0Æ47) 630 (28Æ1) 84 (3Æ7) 852 (40Æ0) Ortiz-Urda et al. Clinically, this patient had multiple aggressive SCCs and died from this complication at the age of 37 years,

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 10–13 Registrars’ Symposium 13

6 years after her first SCC. Our clinicopathological findings, lung, colorectal, breast, cervical and prostate cancer. Using the therefore, suggest that individuals with RDEB can develop SCC Office of National Statistics classification of towns, there was a regardless of type VII collagen expression and that additional marked and highly significant separation between the groups, tumour-promoting events occur. These new data demonstrate with the highest melanoma rates observed in residents of coas- that type VII collagen expression alone is not a reliable indica- tal and countryside towns (15 per 100 000 per year) while the tor of SCC risk in RDEB and that until the cellular events lead- lowest were recorded in districts of inner London (5–9 per ing to SCC invasion are better understood, careful clinical 100 000 per year), (P <0Æ001). Applying the Oxford index of vigilance and early biopsy of suspicious skin lesions remain of multiple deprivation (2004), we found that in contrast to can- paramount importance in the management of patients with cer in general, melanoma showed an inverse correlation with this genodermatosis. deprivation (P <0Æ001). On analysing subdomains of the deprivation index including education, health, crime, environ- ment and income, the strongest correlation in melanoma was RF-7 with high income levels (P <0Æ001). There was no correlation Geographical variation of melanoma rates between rates of melanoma by district with those of lung, in England: preliminary evidence for an prostate or cervical cancer but interestingly, there was a signifi- epidemiological association with breast cancer cant positive correlation of melanoma with breast cancer rates N. Hallai and A.J.G. McDonagh (r =0Æ81, P <0Æ005). As a measure of the influence of local Royal Hallamshire Hospital, Sheffield, U.K. ultraviolet radiation exposure, we grouped the PCT districts by National data for 2001–3 indicate a wide range in the age stan- latitude, demonstrating a weak but significant association of dardized frequency of primary cutaneous melanoma among increasing melanoma incidence with more southerly location English Districts from approximately 3 to 25 per 100 000 per (P <0Æ01). Variations in ethnic mix and ultraviolet exposure annum in men, with a mean of 10.8. Women have higher between districts appeared to explain only a small component mean and maximum rates of 12Æ4 and 34, respectively. The in- of the geographical variation in melanoma rates in England. cidence rates rise progressively with age reaching overall max- Although melanoma is not, in general, associated clinically ima of 41Æ2 in women and 63Æ5 in men over the age of 85. To with breast cancer, common genetic factors or environmental investigate possible determinants of the variation in melanoma influences such as recreational behaviour – perhaps reflecting incidence between districts, we analysed incidence rates in the the correlation observed with income levels – might contribute 303 English primary care trust (PCT) areas for relationship to the correlation we have observed in the occurrence of these with geographical factors and indicators of social deprivation. two malignancies which have both increased in frequency in We also examined the geographical distribution of melanoma recent years. in relation to the commonest adult malignancies including

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 10–13 Clinicopathological Cases: Summaries of Papers

CPC-1 CPC-2 Visceral leishmaniasis presenting cutaneously in Ferguson-Smith type multiple keratoacanthomas Kaposi’s sarcoma with vulval involvement R. Patalay, F. Deroide,* S. Bhagani* and V. Swale* G. Sega-Hall, D. McCormick, S.G. Keohane and The Hammersmith Hospital, London, U.K. and *The Royal Free Hospital, B.R. Hughes London, U.K. St Mary’s Hospital, Portsmouth, U.K. A 40-year-old HIV-positive man who had stopped taking anti- A 79-year-old woman known to have a diagnosis of self-heal- retroviral treatment 5 years previously presented with an 11- ing epitheliomata of Ferguson-Smith, presented with a recent month history of multiple asymptomatic purple over history of two tender nodules located on the left side of the his body. He had lost 2 kg in weight and had night sweats vulva. This patient was well known to our department with a and rigors in the 3 months before presentation. He lived 47-year history of recurrent multiple keratoacanthomas (KAs) mostly in Southern Spain. On examination he had purple pa- and well-differentiated squamous cell carcinomas (SCCs). pules on his left forearm, feet, penis and buccal mucosa. He These lesions appeared on sun-exposed areas. To date she has also had widespread lymphadenopathy and a smooth, non- had approximately 50 of these lesions, which have been trea- tender liver edge. His CD4 count was 39 cells mm–3 and viral ted with various therapies. Untreated lesions tended to resolve load 10 411 copies mL–1. Histology showed a dermal vascular spontaneously. In 1990 she developed a right-sided vulval and proliferation suggestive of Kaposi’s sarcoma (KS) with positive perianal SCC for which she underwent local radiotherapy fol- immunostaining for CD31 and human herpes virus 8. There lowed by wide local excision. Physical examination revealed was an associated peripheral infiltrate of plasma cells and his- two large, red, indurated and dome-shaped vulval nodules. tiocytes. The cytoplasm of the histiocytes contained Leishmania There was no inguinal lymphadenopathy or abdominal amastigotes which were also seen extracellularly. These fea- organomegaly. Histological examination showed that although tures were those of coexistent leishmaniasis and KS. Bone mar- the findings could be consistent with an invasive SCC they row aspirate confirmed visceral leishmaniasis, but polymerase were indistinguishable from self-healing epitheliomata of chain reaction analysis could not determine the species. Leish- Ferguson-Smith. Self-healing epitheliomata of Ferguson-Smith maniasis is caused by protozoa in the genus Leishmania. The are the most common form of multiple KAs. The disorder is clinical presentation varies depending on a number of factors inherited in an autosomal dominant manner; the gene lies in such as the infecting species and the host immunity. There is a narrow region on chromosome 9q (Bale SJ. The ‘sins’ of the a high prevalence of visceral leishmaniasis (VL) among HIV- fathers: self-healing squamous epithelioma in Scotland. J Cutan positive patients in countries around the Mediterranean belt, Med Surg 1999; 3: 207–10). Our patient is originally from particularly in advanced stages of disease (CD4 < 200 mm–3). Scotland where the majority of the cases have been described. In parallel with this are reports of the coexistence of Leishmania All seven of her siblings have been diagnosed with the same parasites in a variety of skin eruptions including KS, dermato- condition. In her case it is difficult to distinguish clinically fibroma, psoriasiform dermatitis, tattoo infiltration, dermato- between SCC and self-healing epitheliomata of Ferguson- myositis and herpes zoster. The role of Leishmania in KS and Smith. The histology is also unhelpful, as even by utilizing the other cutaneous lesions is not entirely clear. It seems unlikely five most useful histological criteria it could not be distin- that the parasites exert a direct pathogenic effect but interest- guished if the lesions were benign squamous proliferations or ingly KS frequently regresses after treatment with appropriate SCCs (Cribier B, Asch P, Grosshans E. Differentiating squa- antiprotozoal therapy. The protozoa have also been isolated mous cell carcinoma from keratoacanthoma using histopatho- from normal skin in patients with HIV and VL suggesting that logical criteria. Is it possible? A study of 296 cases. Dermatology in the immunocompromised host there may be more wide- 1999; 199: 208–12). The use of immunohistological markers spread dissemination. In our patient the finding of Leishmania is controversial. This has created a management dilemma. parasites within KS enabled a diagnosis of unsuspected visceral Because of the site of these lesions, treatment for SCC would leishmaniasis. be more radical and mutilating than for KA. Atypical, or diffi- cult cases, should therefore be considered, and treated as SCCs, as a clear-cut distinction is not possible. There are six reports in the literature of solitary vulval KAs. This is the first report of a patient with Ferguson-Smith type KA developing vulval lesions.

2007 The Authors 14 Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 Clinicopathological Cases 15

CPC-3 CPC-4 Intrafamilial phenotypic variability of dominant and Cutaneous mastocytosis in HIV: an unfortunate recessive dystrophic epidermolysis bullosa resulting coincidence? from a novel glycine substitution, p.G1770S, in type T. McPherson, E. Soilleux,* C. Grills and S. Cooper VII collagen Department of Dermatology, Churchill Hospital, Oxford, U.K. and A. Wong, J.A. McGrath,* R.D. Ead and C. Moss *Department of Cellular Pathology, John Radcliffe Hospital, Oxford, U.K. Birmingham Children’s Hospital, Birmingham, U.K.; *St John’s Institute of A 21-year-old man presented describing an episode of severe Dermatology, London, U.K. and The Royal Oldham Hospital, Oldham, U.K. sunburn while spending time in Thailand the previous year. Dystrophic epidermolysis bullosa (DEB) is characterized by Shortly after recovering from the sunburn, he noticed numer- skin and mucosal fragility, dystrophy and scarring, and is ous red-brown macules appearing over his trunk. Over the caused by mutations in the type VII collagen gene, COL7A1. following year further lesions appeared on the trunk, and to a Severe recessive forms of DEB result from homozygosity or lesser extent, on the upper and lower limbs. The lesions were compound heterozygosity for a variety of loss-of-function occasionally itchy. Examination showed widespread brownish- mutations in COL7A1, while the milder dominant variants of red macules ranging from 0Æ2to0Æ7 cm in diameter. Individ- DEB are usually caused by glycine substitutions in the type VII ual lesions showed a positive Darier’s sign. Punch biopsy collagen triple helix. COL7A1 glycine substitutions have also showed a perivascular and interstitial infiltrate of mast cells, been reported in recessive DEB (RDEB), in asymptomatic het- confirmed by positive immunostaining with mast cell tryptase, erozygous carriers of RDEB, in patients with isolated nail dys- c-KIT (CD117) and CD68 KP1, but not CD68 PGM1. This was trophy, in transient bullous dermolysis of the newborn and in consistent with the clinical diagnosis of mastocytosis. Skin pretibial DEB. We report a pedigree from Pakistan in whom a lesions remained relatively asymptomatic. Haematological novel glycine substitution is associated with severe RDEB analysis of his blood was unremarkable and there was no evi- when present on both alleles but which results in mild dom- dence of systemic involvement. In the year following presenta- inant DEB or no clinical pathology when present on a single tion the patient was diagnosed with HIV. The infection was allele. The probands with typical RDEB are a boy aged 9 presumed to have been contracted while in Thailand as he months in family A, and two brothers aged 1 and 4 years in had had a negative HIV test performed prior to this. HIV sero- family B. None has yet developed the characteristic scarring of conversion has been commonly reported to be accompanied Hallopeau–Siemens RDEB although the oldest child has early by exanthemata consisting of maculopapular lesions distri- webbing and finger contractures. The parents in family A are buted largely on the thorax. Biopsy generally demonstrates a first cousins and are phenotypically normal (skin and nails), nonspecific mononuclear cell infiltrate mainly in the upper with no family history of blisters or nail dystrophy apart from dermis composed of a mixture of CD4+ and CD8+ T lympho- the mother’s niece who has RDEB. By contrast, the parents in cytes with CD68+ histiocytes or dendritic cells. We have been family B, who are second cousins, have familial nail dystro- unable to find reports of cutaneous mast cell infiltration asso- phy: the father and his two brothers have dystrophic big toe ciated with HIV seroconversion. While there are no specific nails while the mother and her two sisters have dystrophy of reports regarding the interactions between HIV and mast cells, all finger and toe nails. In addition to their two sons with there are several ways in which basophils, a closely related cell RDEB, they have two daughters with the father’s pattern of have been seen to interact. Firstly, the HIV transactivator (Tat) nail dystrophy confined to the toe nails. Furthermore, the protein acts as a strong chemoattractant for FcRI-positive cells mother also has pretibial lichenoid lesions, not reported in by means of its interaction with the chemokine receptor any other family members. The two families A and B are rela- CCR3. Secondly, peptides from the HIV gp41 envelope glyco- ted through their maternal grandfathers who are cousins. Skin protein are also chemotactic for basophils. Finally, basophils biopsy in the affected RDEB children showed sublamina densa express a number of HIV receptors on their surface, including cleavage at the dermal–epidermal junction with markedly CD4, CCR3, CCR5 and CXCR4, with HIV Tat protein able to reduced labelling for type VII collagen on immunofluores- upregulate CCR3. Accordingly, HIV infection of basophils has cence microscopy. Transmission electron microscopy showed been reported. Therefore, a number of mechanisms might be only sparse and wispy anchoring fibrils. Mutation analysis operative to attract basophils or to mediate their infection with identified a previously unreported glycine substitution in HIV. Whether analogous processes may occur involving mast COL7A1, p.G1770S, which was homozygous in the children cells is unknown. As far as we are aware, the association of with RDEB and heterozygous in the parents. This extended HIV and mastocytosis has not previously been reported. Our family shows that a single glycine substitution in COL7A1 can case demonstrates an interesting temporal relationship result in several different phenotypes with heterozygous carri- between the development of cutaneous mastocytosis and ers ranging from clinically normal, through isolated toe nail recent HIV infection. dystrophy to dystrophy of all nails, with or without pretibial lesions. Clearly, the protean clinical expression associated with p.G1770S on one allele indicates that the dominant DEB phe- notype may be influenced by other as yet undisclosed genetic or nongenetic factors.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 16 Clinicopathological Cases

CPC-5 CPC-6 Annular variant of bullous congenital ichthyosiform Mild familial Conradi–Hunermann–Happle syndrome erythroderma with a novel mutation in keratin 10 presenting with naevus superficialis lipomatosus and flare during pregnancy M. Barkham, M.A. Brundler and C. Moss N. Sheth, D. Greenblatt, F. Teixeira and Birmingham Children’s Hospital, Birmingham, U.K. J.A. McGrath* Conradi–Hunermann–Happle syndrome (CHH) is a distinctive Ealing Hospital, London, U.K. and *St John’s Institute of Dermatology, X-linked dominant disorder characterized by linear London, U.K. and pigmentary change, follicular atrophoderma and cicatricial Bullous congenital ichthyosiform erythroderma (BCIE) is a alopecia. It is one of a group of conditions called chon- rare autosomal dominant disease of cornification associated drodysplasia punctata, characterized by punctate epiphysial with mutations in keratin genes 1 and 10. We report the sev- stippling. CHH is often associated with skeletal defects, short enth known family with a rare variant consisting of scaly stature and congenital cataracts. We report a girl in whom fa- annular erythematous plaques. A 43-year-old Pakistani woman milial CHH was a coincidental finding. This girl presented had erythroderma at birth and blisters in childhood which aged 6 months with a soft, lumpy, flesh-coloured birthmark gradually improved. By her 30s she had only annular erythe- on the right flank, in a segmental distribution. Streaky hypo- matous hyperkeratotic plaques on the extremities and flexures. pigmentation on the trunk was noted coincidentally. A diag- Her disease was usually mild but occasionally the plaques nostic biopsy was planned, but she was lost to follow-up until became painful and inflamed, especially during the second tri- age 7 years. Examination then revealed ichthyosis on the mester of each of her two pregnancies. During these flares the lower legs and dorsum of feet, with extensive linear hypo- plaques became more extensive in distribution coalescing over pigmentation and follicular atrophoderma following Blaschko’s large areas. They improved spontaneously soon after delivery. lines, in addition to the birthmark which was unchanged. She She was the first affected member of her family. Her first son was otherwise growing and developing normally, with no was unaffected but her younger son also developed blisters overt skeletal abnormalities or visual problems. Her mother after birth. His condition improved: by age 16 he had only a had similar streaky skin lesions and scarring alopecia, and few dry scaly plaques over his body. A skin biopsy from a pla- reported that two maternal aunts have similar skin problems. que in the mother and affected son showed hyperkeratosis, A clinical diagnosis of CHH was made on the basis of the lin- acanthosis, hypergranulosis, prominent hyaline granules and ear skin changes and family history. A skin biopsy from the cytoplasmic vacuolization in the superficial epidermis. Muta- lumpy birthmark showed adipose tissue extending into the tion analysis on genomic DNA isolated from peripheral blood superficial dermis, consistent with naevus superficialis - of the proband, affected son and 50 unrelated healthy individ- tosus. There was also with ruptured hair follicles. uals revealed no mutation in K2e or K1 but a Q447P mutation DNA analysis revealed heterozygosity for a novel missense in the 2B segment of K10. This was found in both affected mutation in exon 2 of sterol isomerase emopamil binding patients but not in any of 100 normal K10 alleles screened, protein, confirming CHH (Molecular Genetics Laboratory, Exe- suggesting it is the mutation causing the disease. This was ter, U.K.). Phenotypically mild CHH, as seen in our patient, consistent with a diagnosis of BCIE. Among other families can be caused by skewed X-inactivation, but that explanation with the annular variant the most limited cases to date, like is less likely here as all family members apparently share the those of our family, had a mutation in the helix termination mild phenotype. Future genotype–phenotype studies may motif of keratin 10 suggesting that mutations in this region show that this mutation predicts a mild phenotype. Naevus may result in the mildest phenotype (Suga Y, Duncan KO, He- superficialis lipomatosus has not previously been described in ald PW, Roop DR. A novel helix termination mutation in kera- association with CHH. The association of two such rare condi- tin 10 in annular epidermolytic ichthyosis, a variant of tions is unlikely to have occurred by chance, and the segmen- bullous congenital ichthyosiform erythroderma. J Invest Dermatol tal pattern and follicular abnormality within the fatty naevus 1998; 111: 1220–3). Worsening of BCIE in pregnancy has suggest a pathogenetic link with CHH which particularly af- not been previously reported. There is no evidence of any fects hair follicles with scarring alopecia and follicular known direct hormonal effects on intermediate filament struc- atrophoderma. In the light of this diagnosis, the family will ture or function. Perhaps the most likely explanation for this be offered genetic counselling and ophthalmological examin- is related to the temperature instability of the cell cytoskele- ation. The subtle clinical signs of CHH in this girl show how ton. Studies on epidermolysis bullosa simplex (EBS) cell lines easily this diagnosis can be overlooked. show that after thermal stress, keratin filaments became unsta- ble breaking into aggregates similar to those seen in the skin of patients with EBS. In pregnancy the baseline temperature increases 0Æ5–1Æ0C during the second trimester. Therefore epidermal fragility maybe exacerbated during this period in patients who already have a compromised cell cytoskeleton.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 Clinicopathological Cases 17

CPC-7 CPC-8 A rare case of bromoderma and bromism Atypical with fibrillar IgA J. Hafiji, V. Majmudar,* S. Matthews,* E. Rytina and deposition N.P. Burrows S.E Clements, C.M. Stefanato, B. Bhogal and Addenbrooke’s Hospital, Department of Dermatology, Cambridge, U.K. and R.W. Groves *Mid Essex Hospitals NHS Trust, Department of Dermatology, Essex, U.K. St John’s Institute of Dermatology, London, U.K. A 56-year-old woman with polycythaemia vera (PCV) presen- Dermatitis herpetiformis (DH) is a chronic papulovesicular ted with a 2-month history of pruritic, tender erythematous disorder that is characterized immunohistologically by IgA nodules affecting predominantly her forehead, upper trunk deposition in the papillary dermis. Granular IgA is the classical and limbs. Four months before this, she was hospitalized with pattern seen in DH, and although fibrillar and continuous psychotic depression. Her PCV had been recalcitrant to stand- granular patterns are also recognized, they are extremely rare. ard treatment and she was therefore commenced on pipobro- A 53-year-old man with a history of DH that had been quies- man (Vercite; Abbott S.p.A, Campoverde di Aprilia (LT), cent for 10 years on dapsone 50 mg twice daily, presented Italy) 75 mg once daily which she had been taking for the with an acute flare of his disease. Clinical examination previous 7 months. Examination revealed a symmetrical revealed widespread annular urticated plaques with vesicles erythematous, papulonodular eruption with erosions and pus- and bullae clustered at the periphery. The pruritic eruption tulation. Smaller haemorrhagic lesions with necrotic-looking progressed over the following week to involve his face, lips, areas were also noted on the pulps of several fingers. Laborat- hands and feet with development of erythematous targetoid ory investigations showed a mildly elevated white blood cell lesions on both palms. His clinical presentation was atypical count, raised inflammatory markers and a serum bromide level for DH and a diagnosis of linear IgA disease was also consid- of 48Æ8mgL-1 (upper limit of normal < 10 mg L-1). Perinu- ered. Histology from lesional skin revealed a neutrophil-rich clear antineutrophil cytoplasmic antibodies were positive, but subepidermal with scattered eosinophils. Direct immu- antinuclear antibodies, double-stranded DNA and complement nofluorescence of two nonlesional skin biopsies showed a levels were normal. Skin swabs were negative and a computed fibrillar IgA pattern within the papillary dermis. Indirect im- tomographic scan of her brain was normal. Histology revealed munofluorescence was negative, and a coeliac antibody screen a normal epidermis but an extensive neutrophilic infiltrate along with two duodenal biopsies confirmed an absence of within the dermis, forming . Gram and periodic gluten-sensitive enteropathy. Human leucocyte antigen (HLA) acid–Schiff stains for microorganisms were negative. The pip- typing revealed the HLA DQ2 antigen which is commonly obroman was discontinued and the patient was placed on a seen in individuals with DH. Dapsone 50 mg twice daily was high sodium chloride and fluid diet and treated topically with continued and, with additional prednisolone 30 mg daily, his clobetasol propionate (Dermovate-NN; GlaxoSmithKline, skin improved dramatically after 2 weeks. We subsequently Uxbridge, U.K.). There was complete resolution within reviewed the records of 600 patients who had shown positive 8 weeks and during this period, her mental state also returned direct immunofluorescence for DH in our department between to normal. Halogenodermas are rare dermatoses that develop 1994 and 2004. Of these 600 individuals, only 12 had a following exposure to bromides, fluorides and iodides. Halo- fibrillar pattern of IgA deposition. Interestingly, these 12 gens may induce acneiform eruptions, pustules and, less com- patients had a wide variety of clinical presentations, many of monly, granulomatous or vegetative plaques, ulcers and even which were atypical for DH. Suggested diagnoses included lin- bullae. Cutaneous lesions often appear after long-term expos- ear IgA disease (five patients), (two ure and can be associated with bromism; a syndrome charac- patients), erythema multiforme (one patient) and nodular vas- terized by weakness, restlessness, headache, ataxia and culitis (one patient). Fibrillar IgA is an extremely unusual pat- personality changes (Carney MW. Five cases of bromism. Lancet tern of deposition seen in a minority of individuals with DH. 1971; ii: 523–4), as seen in this case. Some authors have sug- The reason for this appearance is unclear. Interestingly, only gested that halogenodermas represent hypersensitivity reac- 34 individuals have been diagnosed with DH in Japan. Of tions (Rosenberg FR, Einbinder J, Walzer RA, Nelson CT. these, a high proportion (56%) have a fibrillar IgA pattern of Vegetating iododerma. An immunologic mechanism. Arch Der- deposition, with an associated low incidence of gluten-sensi- matol 1972; 105: 900–5); however, accumulation of the halo- tive enteropathy and absence of the HLA-B8/DR3DQ2 haplo- genide is an important factor. The mainstay of treatment is a type associated with DH in white people. Our study highlights high salt and fluid intake, topical and systemic steroids the possibility that the fibrillar IgA pattern may represent together with diuretics but in severe cases immunosuppressive patients with an atypical clinical presentation of DH, and in agents such as ciclosporin (Novartis Pharmaceuticals, Frimley, conjunction with the Japanese data we speculate that it may U.K.) have been used. This case illustrates the classical features represent a separate clinical subtype of the disease. of a dermatosis that is rarely encountered by the dermatolo- gist.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 18 Clinicopathological Cases

CPC-9 CPC-10 Adalimumab-induced subacute cutaneous lupus Protein C deficiency as a rare cause of diffuse dermal erythematosus angioendotheliomatosis N. Sheth, D. Greenblatt, S. Patel* and K.M. Acland M. Eldarouti, A. Zidan, A. Dakhly and M. Abdelmalek Ealing Hospital, London, U.K. and *Newham General Hospital, London, Cairo University Teaching Hospitals, Cairo, Egypt U.K. Diffuse dermal angioendotheliomatosis (DDA) is a rare variant Adalimumab is a human monoclonal antibody to tumour of reactive angioendotheliomatosis (RA), which is associated necrosis factor (TNF)-a. It is a treatment option for rheu- with a number of systemic diseases. We report a case of pro- matoid arthritis. All TNF-a antagonists have been associated tein C deficiency presenting as DDA. A 2-year-old boy presen- with lupus autoantibodies. The development of drug-induced ted with a 7-month history of gradual onset and progressive lupus erythematosus is, however, rare. We report a 51-year- course of violaceous, reticulate and haemorrhagic patches, pla- old white woman who developed a rash after starting adal- ques, irregular ulcers covered with black eschars and scars at imumab for seropositive rheumatoid arthritis mainly affecting the sites of old healed ones. Lesions were distributed over the the small joints of her hands. There was no extra-articular lower face, neck, chest, abdomen and buttocks. The differen- involvement. Her brother had systemic lupus erythematosus tial diagnosis was antiphospholipid syndrome, protein C and S (SLE) with renal and cutaneous involvement. Her arthritis had deficiency or cryoglobulinaemia. The following investigations initially been controlled by methotrexate but this induced were done: full blood count, antinuclear antibodies, protein C rheumatoid nodules. Hydroxychloroquine and sulfasalazine and S levels, anticardiolipin, lupus anticoagulant and cryoglob- were not tolerated because of side-effects. In summer 2005, ulin levels. Skin biopsies were taken from the edge of the adalimumab was started at a dose of 40 mg subcutaneously ulcers and stained with haematoxylin and eosin. Results on alternate weeks. Ten days later the arthritis improved but showed a low protein C level of 46Æ7% (normal 70–140%), she developed a pruritic erythematous rash in a photosensitive with all other laboratory investigations being normal. Skin distribution on her arms and chest with sparing of the face. biopsies revealed diffuse proliferation of endothelial cells in She had no systemic symptoms. There was no evidence of between the collagen bundles of the reticular dermis. Immu- neurological involvement or serositis. Following a second nohistochemical studies on paraffin sections treated with dose, the rash worsened and adalimumab was stopped. A skin CD34 monoclonal antibodies revealed positive cytoplasmic biopsy showed a dense perivascular lymphohistiocytic infil- staining for tumour cells by chromagen. The final diagnosis of trate, hydropic change along the basal layer and occasional our case was protein C deficiency presenting as DDA. We can apoptotic cells. This was consistent with subacute cutaneous conclude that protein C deficiency can be considered as a rare lupus erythematosus (SCLE). Direct immunofluorescence of le- cause of DDA. sional and uninvolved skin were both negative. Antinuclear factor antibody (ANA), which had been negative prior to treatment, became positive (1 : 80, homogeneous pattern). Double-stranded DNA, extractable nuclear antigens, C3/C4 and antihistone antibodies were all negative. All other tests CPC-11 were unremarkable. The patient fulfilled only two of the 11 A rare case of cutaneous lymphomatoid criteria of the American College of Rheumatology for SLE. granulomatosis: demonstrating the importance of Two weeks after stopping the adalimumab the rash resolved immunophenotyping and molecular techniques without treatment and ANA became negative again. There are E. Hiscutt, M.J. Galloway, G.J. Gibson* and few case reports of cutaneous lupus erythematosus following S. Natarajan anti-TNF-a treatment. Most are associated with a low ANA Sunderland Royal Hospital, Sunderland, U.K. and *Freeman Hospital, titre and resolve on discontinuation of the drug. The mechan- Newcastle-Upon-Tyne, U.K. ism of lupus-like syndromes with TNF-a antagonists is uncer- A 40-year-old man presented with shortness of breath, cough tain. Adalimumab induces apoptosis prompting the release of and anaemia. He was diagnosed with lymphomatoid granulo- nuclear antigens. The engagement of rheumatoid-factor matosis (LG) after open lung biopsy; imaging revealed add- expressing B cells and toll-like receptor-9 with these agents itional involvement of the kidneys, spleen and brain. Initial results in antibody formation. These form immune complexes treatment was with interferon (IFN)-a; however there was causing end-organ damage. Clinical manifestations are, how- rapid disease progression. Further chemotherapy (cyclo- ever, rare as antibody formation is just one part of the aetio- phosphamide, doxorubicin, vincristine, prednisone [CHOP], pathogenetic stages in the development of lupus rituximab and intrathecal methotrexate) resulted in remission erythematosus. There perhaps needs to be a genetic predispo- both clinically and radiologically. One month after cessation sition. Environmental eliciting factors, such as ultraviolet radi- of therapy asymptomatic, cutaneous nodules developed and ation exposure in our patient, may also be required. This is gradually increased in size and number. Examination revealed the first reported case of adalimumab inducing SCLE. Although multiple papules and nodules on the trunk, neck and scalp, generally a well-tolerated agent, such reactions may occur which were both subcutaneous and dermal in location. Colour more frequently with its increasing use. ranged from pink to light brown. Histology revealed diffuse

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 Clinicopathological Cases 19 infiltration of full-thickness dermis and subcutis, with lym- (man, 25 years) minocycline; case 4 (man, 62 years) phenyt- phohistiocytic inflammatory infiltrate. There was perivascular oin or carbamazepine. The delay between introduction of and periappendiceal involvement, with a focal angioinvasive causative drug(s) and onset of rash or systemic features was pattern. Cutaneous clinical features have regressed with the 20–49 days (mean 27 days). Cases 1 and 4 developed a current treatment of IFN-a, 3 mega units, three times weekly. morbilliform eruption, case 2 a widespread urticated erythema LG was first described in 1972 (Liebow AA, Carrington CR, and case 3 a polymorphic rash with eczematous papules, atyp- Friedman PJ. Lymphomatoid granulomatosis. Hum Pathol 1972; ical targets and pustules. All four patients displayed facial 3: 457–558). It was described as a unique angiocentric and swelling, fever and cervical lymphadenopathy. All patients had angiodestructive lymphoproliferative process, occurring in the eosinophilia [mean maximal eosinophilia 1Æ72 x 109 L–1 (nor- lungs. For almost 20 years the aetiology was unknown and LG mal <0Æ4x109 L–1)]. All patients had abnormal liver transam- remained an enigma. Due to the prominent reactive T-cell inases [mean maximal aspartate aminotransferase 140 IU L–1 infiltrate it was thought to be perhaps a T-cell lymphoprolifer- (normal 10–50 IU L–1)]. Skin biopsy showed histopathologi- ative disorder. Several molecular techniques have been used to cal features indicative of a in all cases. The elucidate the aetiology of LG and distinguish it from other causative drug was discontinued at a mean of 29 days after pathology, such as extranodal natural killer or T-cell lymph- initiation (range 22–53 days). Cases 1, 2 and 3 received oral oma. Immunohistochemical studies using panels of antibodies corticosteroids initially (1 and 3, prednisolone 30 mg daily; have characterized the infiltrate of LG. It is composed of infre- 2, dexamethasone 2 mg three times a day); however, the clin- quent B cells and numerous reactive T cells, which are usually ical features, eosinophilia and transaminases, continued to CD3 and CD4 positive. Most atypical lymphoid cells express deteriorate and so they were given intravenous methylpred- CD20, indicating B-cell phenotype. Polymerase chain reaction nisolone 500 mg on three consecutive days. Case 4 was given (PCR) and in situ hybridization have located Epstein–Barr virus methylprednisolone 1 g on three consecutive days without (EBV) RNA to B cells within some tumours of LG. This has preceding oral corticosteroid. In all patients the rash, lympha- helped characterize it as an EBV-positive B-cell lymphoprolif- denopathy, fever and blood test abnormalities resolved swiftly. erative disease. PCR antigen receptor gene rearrangement an- Following methylprednisolone the patients were given oral alysis has demonstrated clonal immunoglobulin heavy chain prednisolone 30 mg daily, reducing to zero over the next 10– (IgH) and rarely T-cell receptor (TCR) gene rearrangements, 40 days (mean 23 days). Case 4 developed a bacteraemia fol- in some patients. In our case, molecular studies (lung) lowing treatment with methylprednisolone. On review at 40 showed negative IgH, a clonal Igj peak and 90% polyclonal T days post-methylprednisolone all patients were well with mild cells with a very small clonal peak for TCR. All these tech- skin dryness and normal or minimally raised eosinophil count niques have been essential in determining the aetiology of LG (mean 0Æ43 x 109 L–1) and normal or minimally raised trans- and developing targeted therapy such as rituximab. aminases (mean 59 IU L–1). These results indicate that the use of intravenous methylprednisolone in the treatment of DRESS is an effective and safe method of inducing a rapid remission in a potentially life-threatening illness.

CPC-12 Rapid and sustained response to intravenous methylprednisolone in four cases of DRESS J. Natkunarajah, S.K. Goolamali, K. Watson, CPC-13 R. Morris-Jones, C.S. Macfarlane, E.M. Higgins, Targeted monoclonal therapy in hypereosinophilic R.A. Marsden* and J.D. Creamer syndrome Department of Dermatology, King’s College Hospital, London, U.K. and R. Patalay, D. Milojkovic, A.C. Chu and F. Teixeira *Department of Dermatology, St George’s Hospital, London, U.K. The Hammersmith Hospital, London, U.K. DRESS (drug rash with eosinophilia and systemic symptoms) The patient presented aged 19 with a maculopapular rash and is a severe, drug-induced hypersensitivity syndrome in which an isolated eosinophilia. Over the following 5 years he had a a widespread eruption is associated with multiorgan involve- fluctuant maculopapular rash, pruritus and an eosinophil ment, most notably liver dysfunction. Corticosteroids are the count between 20 and 25 x 109 L–1. Further investigations first-line treatment in DRESS; however, there are no controlled over this period found no underlying cause for his eosino- trials or published guidelines to indicate the optimal route, philia but did isolate a peripheral T-cell clone. He was diag- dosage or length of steroid therapy. Oral steroids can be asso- nosed with the lymphocytic variant of hypereosinophilic ciated with poor response or with disease relapse when the syndrome. He experienced two major flares which were min- dose is tapered. We present four patients diagnosed with imally responsive to oral prednisolone, hydroxyurea and DRESS who all showed a rapid and sustained response when imatinib (tyrosine kinase inhibitor). However, he did have a treated with intravenous methylprednisolone. The culprit good, but short-lived response, to alemtuzumab (monoclonal drugs in the four cases were: case 1 (woman, 19 years) sulfa- anti-CD52 antibody). In June 2006, during a third flare, the salazine; case 2 (woman, 40 years) carbamazepine; case 3 anti-interleukin (IL)-5 monoclonal antibody mepolizumab was

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 20 Clinicopathological Cases used. Within a week the skin infiltrate had fully resolved, the diagnosis of TEN. Thalidomide was stopped and support- there was a great improvement in the pruritus and the eosino- ive skin care instituted. Despite this she continued to deterior- phil count fell dramatically. Over the following 6 months, ate and died. Case 2 was a 64-year-old man who had been with repeated infusions, the eosinophil count remained stable diagnosed with multiple myeloma. He agreed to participate in but the maculopapular rash and pruritus gradually returned, to a phase II randomized control trial of treatment of multiple which he had some response to ciclosporin. Hypereosinophilic myeloma. He was randomized to the thalidomide treatment syndrome is uncommon and defines a group of similar idio- group. Six weeks later he developed a generalized erythema- pathic conditions that are unified by a hypereosinophilia and tous rash which prompted the haematologists to stop thalido- disseminated end-organ involvement in the absence of an mide immediately. Over the next 4 days he was referred to underlying cause. The condition has recently been subclassi- dermatology with worsening of this rash. Examination fied into myeloproliferative and lymphocytic variants, reflect- revealed a widespread dusky erythematous rash, with areas of ing the aetiology of the underlying eosinophilia, still leaving a desquamation and blistering. There was widespread involve- true idiopathic hypereosinophilic syndrome. The most com- ment of the trunk and limbs. Biopsy confirmed TEN. He was mon organ to be affected is the skin, with up to 50% of admitted to the burns unit for supportive care. Over the next patients having mucocutaneous symptoms. Almost all patients 48 h his skin started to improve. He had no long-term com- with the lymphocytic variant have cutaneous manifestations. plications. are common in patients on thalidomide for Twelve per cent of patients are asymptomatic at presentation. multiple myeloma (Hall VC, El-Azhary RA, Rajkumar SV. Der- The T-cell clone in the lymphocytic variant secretes IL-5 matologic side effects of thalidomide in patients with multiple which is the main cytokine responsible for the eosinophilia. myeloma. J Am Acad Dermatol 2003; 48: 548–52). Despite TEN End-organ damage is rare and the short-term prognosis is being a rare complication, prompt recognition of this poten- good in this variant. However, the long-term prognosis is less tial adverse effect with immediate discontinuation of thalido- favourable due to the occurrence of T-cell malignancies. New mide is very important. With increased use of thalidomide, targeted biological treatments have both increased our under- this potentially fatal complication may be more frequently standing of this condition at the molecular level and our abil- encountered by dermatologists in future. ity to treat these patients effectively.

CPC-15 CPC-14 Radiotherapy: a protective role for toxic epidermal Toxic epidermal necrolysis complicating treatment necrolysis? with thalidomide for multiple myeloma J. Hafiji, S. Orpin* and H. Lewis J. Angus, A. Affleck, I. Leach and J.L. Bong Addenbrooke’s Hospital, Cambridge, U.K.; *Heart of England NHS Trust, Queen’s Medical Centre, Nottingham, U.K. Birmingham, U.K. and University Hospital Birmingham NHS Trust, With advances in the treatment of multiple myeloma, thalido- Birmingham, U.K. mide is increasingly becoming a routine therapeutic option in Toxic epidermal necrolysis (TEN) is a rare medical emergency, both relapsed and treatment-unresponsive disease, and more first described by Lyell approximately 60 years ago, character- recently is also being used as a first-line agent. Toxic epider- ized by extensive epidermal cell death. Drugs are thought to mal necrolysis (TEN) is a recognized complication of thalido- be the trigger in virtually all cases, but the precise sequence of mide. We present two cases developing TEN after receiving molecular and cellular events leading to the development of thalidomide for myeloma and highlight the importance of TEN is only partially understood. We present a case of TEN early recognition in prognosis. Case 1 was a 77-year-old where radiotherapy appears to have had a protective role. A woman who was diagnosed with multiple myeloma following 49-year-old man with a history of metastatic rhabdomyosarco- a pathological fracture of the right femur. She had a poor ma was admitted with pneumonia and treated with intraven- response to initial treatment with melphalan and prednisolone ous co-amoxiclav (Augmentin; GlaxoSmithKline, Uxbridge, and so was commenced on cyclophosphamide, thalidomide U.K.) and levofloxacin (Tavanic; Sanofi-Aventis, Guildford, and dexamethasone. There was a good response to treatment U.K.) for 5 days. A week later he developed a tender, erythe- which was discontinued. However, 8 months later she was matous eruption in his groins and lower abdomen which admitted with lethargy and general deterioration with evi- evolved over the next 48 h into erythroderma and extensive dence of disease relapse. She was restarted on thalidomide. Six blistering affecting approximately 50% of his body surface weeks later, she was readmitted with a generalized rash, fur- area. A large sharply demarcated rectangular area of skin on ther deterioration, increasing confusion and worsening renal his chest and back was entirely unaffected. This was the site function. The rash became more extensive with mucosal of radiotherapy which he had 3 months previously for super- involvement. She was referred to dermatology. Examination ior vena cava obstruction from pulmonary metastases. Laborat- revealed confluent patches of erythema with overlying desqua- ory investigations showed a normal white blood cell count mation. The Nikolsky sign was positive. Histology confirmed with lymphopenia, anaemia, significantly raised inflammatory

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 Clinicopathological Cases 21 markers, low albumin, reduced bicarbonate level but normal was given every 8–12 weeks for eight sessions. Further cour- urea and glucose. While skin swabs were negative, blood cul- ses of laser treatment were subsequently administered to pla- tures were positive for enterococci and coagulase-negative sta- ques on the thighs, groins, upper back and scalp. The phylococci. A diagnostic biopsy revealed a subepidermal split treatment was painful but tolerable and all wounds healed with full-thickness epidermal necrosis. The patient was resus- well without complications and only minimal scarring. Objec- citated, all nonessential drugs were discontinued and he was tive and complete symptomatic improvement has been sus- treated with intravenous immunoglobulin (Vigam; Bio Prod- tained in the treatment areas for up to 7 years. Features of ucts Laboratory, Elstree, U.K.) 1 g kg–1 daily for 5 days Darier’s disease have persisted in areas peripheral to the treat- together with intravenous meropenem (Meronem; Astra-Zen- ment site. A skin biopsy from a laser-ablated area 3 years after eca, So¨derta¨lje, Sweden) and vancomycin (Vancocin; Flynn treatment showed no histological features of residual Darier’s Pharma Ltd, Hitchen, U.K.). Initially, he was managed on the disease. The mechanisms by which laser treatment improves burns unit but subsequently required intensive care support. Darier’s disease remain to be elucidated. It may reduce the Our patient made an excellent recovery and was able to return irritancy from sweating by destroying sweat glands or it may home 8 days later. As his skin returned to normal, the rectan- inhibit the production of acantholytic lesions by obliterating gular areas on his chest and back were no longer visible. The hair follicles. Laser ablation of Darier’s disease has been pathogenic mechanisms of this condition are unclear. TEN is a detailed in two previous publications. Each study successfully human leucocyte antigen class I-restricted drug hypersensitiv- treated two patients; the first study utilizing CO2 laser, the ity reaction leading to clonal expansion of CD8+ cytotoxic second the erbium : yttrium aluminium garnet (Er : YAG) lymphocytes (CTLs) only (Nassif A, Bensussan A, Boumsell L laser. Our report adds to these limited data, suggesting that et al. Toxic epidermal necrolysis: effector cells are drug-specific laser treatment of Darier’s disease may be efficacious in severe cytotoxic T cells. J Allergy Clin Immunol 2004; 114: 1209–15). cases. Cytotoxicity appears to be mediated by CTL granzyme, Fas lig- and and tumour necrosis factor. Although there are reports of radiotherapy being a potential risk factor for developing TEN (Trent JT, Bowes LE, Romanelli P, Kerdel FA. Toxic epidermal CPC-17 necrolysis of the scalp following anticonvulsant use and cranial maligna – outcomes of treatment with irradiation. J Cutan Med Surg 2001; 5: 475–8), to our know- Q-switched Nd:YAG and Alexandrite lasers ledge, this is the first case where radiotherapy appears to have V. Madan and P.J. August had a protective role. We hypothesize that this may be a result Hope Hospital, Manchester, U.K. of inactivation of cytotoxic T lymphocytes, a key player in the Surgical excision is considered the gold standard treatment for aetiology of this rare, devastating condition. lentigo maligna. However, this option may sometimes be unsuitable for the very frail and elderly patients or may be declined. We evaluated the role of Q-switched Nd:YAG and Alexandrite lasers in the treatment of lentigo maligna. Q- switched Nd:YAG and Alexandrite lasers were used to treat CPC-16 histologically proven lentigo maligna in 22 patients who were Darier’s disease successfully treated with laser either considered unsuitable for wide surgical excision or ablation declined it. Treatments were continued until clinical cure was V. Brown, S.M. Burge, S.E. Kelly* and N.P.J. Walker achieved. Further treatments were given for clinical recur- Department of Dermatology, Churchill Hopsital, Oxford, U.K. and rences. Patients were assessed at 6 monthly intervals for a *Department of Dermatology, Royal Shrewsbury Hospital NHS Trust, maximum period of 5 years. Complete clinical response was Shrewsbury, U.K. achieved in 12 patients after one to four treatments and a fol- Extensive, severe or recalcitrant Darier’s disease represents a low-up of 1Æ2–5 years after last treatment. Nodular melanoma therapeutic challenge. Anecdotal reports recommend surgical developed in two lesions. After a 50% improvement no fur- removal of lesions using a variety of methods. Laser resurfac- ther response was seen despite six treatments in another ing is one option that allows a highly controlled, selective patient. Partial response to treatment was seen in one patient ablation of the skin, with significant advantages over other and recurrence was noted in four patients. One patient was destructive techniques, such as dermabrasion or electrosurgical lost to follow-up and another who had no recurrence at excision. A 45-year-old man with longstanding widespread, 1-year follow-up died of unrelated causes. After a partial erosive Darier’s disease was experiencing significant pain, and response one patient opted for surgical excision. Excellent cos- physical and social hardship due to his skin disorder. He had mesis was achieved in all patients. Although Q-switched been treated with oral retinoids for more than 15 years and, Nd:YAG and Alexandrite laser treatment of lentigo maligna but still required intermittent systemic antibiotics and antiviral produces a variable clinical response, the ease and speed of therapy for secondary skin infections. In 1999, treatment with this treatment and cosmetic outcomes achieved, make these a the CO2 laser was commenced, initially to the large affected suitable alternative to surgical excision, especially in the frail areas over the central abdomen and lower back. Treatment and elderly or in those patients who refuse surgical treatment.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 22 Clinicopathological Cases

CPC-18 tous reaction. All investigations including a connective tissue Dermal fillers for wrinkling – adverse effects screen and test for sarcoidosis were negative. The patient was presenting to a dermatologist treated with a 2-week course of oral steroids with excellent K. Baxter, M. O’Connell,* D. Scott and results. Improvement was assessed by serial photography and A.M. Layton* a novel examination using the SIAscope (spectrophotometric Foundation Skin, *Department of Dermatology and Department of Histopa- intracutaneous analysis). This is a noninvasive multispectral thology, Harrogate and District NHS Foundation Trust, Harrogate, North scanning technique from which it is possible to derive micro- Yorkshire, U.K. architectural information from the skin. The dermal filler There has been a huge increase in the demand and exploita- Restylane is biodegradable. Thus, the incidence of this sort tion of cosmetic techniques in dermatology over the past dec- of reaction is very unusual but not unprecedented (Lombardi ade. This rapid growth will lead to dermatologists being T, Samson J, Plantier F et al. Orofacial granulomas after injec- increasingly faced with managing the adverse effects of such tion of cosmetic fillers. Histopathologic and clinical study of treatments. We report a case of orofacial granulomas in a 39- 11 cases. J Oral Pathol Med 2004; 33: 115–20). This case dem- year-old woman who had undergone Restylane (Q-Med AB, onstrates the clinical appearance of the granulomas following Uppsala, Sweden) injections for wrinkling. Our patient had the administration of dermal fillers. It also demonstrates a received three treatments around the nasolabial folds and lips novel technique for assessing granulomatous changes with the in August and November 2004 and August 2005. She devel- SIAscope and describes the treatment required to resolve the oped minimal erythema and no inflammation or tenderness problem. We take the opportunity to review the clinical prob- post-treatment. However, approximately 8 months after the lems that might be encountered with the administration of final injection the patient noticed increased thickness, indura- dermal fillers in aesthetic dermatology. This has increasing tion and grey mottling of the skin. Thee changes were con- clinical relevance, as the use of dermal fillers becomes more fined to the treatment areas. Clinically, dermal fibrotic plaques widespread. This presentation represents a practical guide for were evident and these were in keeping with granulomas. A dermatologists who might encounter these problems. biopsy taken from the affected skin confirmed a granuloma-

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 14–22 Bristol Cup Posters: Summaries of Posters

P-1 abnormality. Conclusion: This combination is very effective Combination of low-dose isotretinoin and oral even in very severe acne. Side-effects were minor and could be azithromycin pulse in treatment of moderate to managed conservatively. The small number of patients, short severe acne vulgaris: a preliminary open single arm post-treatment follow-up and open study were the main limi- prospective study tations. D. De and A. Kanwar Postgraduate Institute of Medical Education and Research, Chandigarh, India Background: Both isotretinoin and azithromycin are effective in the treatment of moderate to severe acne vulgaris. How- P-2 ever, recommended doses of isotretinoin are associated with Treatment-seeking and adherence behaviour in distressing side-effects that lead to decreased compliance. patients with acne Azithromycin is relatively well tolerated amongst the anti-acne J. Hassan and V. Yates antibiotics. The efficacy and safety of a combination of these Salford Royal Hospitals Trust, Manchester, U.K. agents have not been studied. Aims and objectives: To assess Acne has been associated with negative psychological effects the efficacy and safety profile of a combination of low-dose including poor mental health, low self-esteem and poor social isotretinoin and oral azithromycin pulse in treatment of mod- functioning. For the suppression of acne and prevention of erate to severe acne. Materials and methods: Patients with scarring and psychological distress, early and adequate treat- Grade 3 or 4 acne, according to the FDA global score, were ment is necessary. However, research suggests that patients included. Any patients with a contraindication for isotretinoin may delay seeking medical treatment for acne and suffer for or those who could not come for the proposed regular fol- years before receiving effective therapy or being referred to a low-up visits were excluded. The baseline haemogram, liver specialist. Additionally, adherence rates to acne treatment function test and lipidogram along with a pregnancy test regimes have been reported as low as 12% rising to 49%. This where applicable were done. Isotretinoin was given in a dose research aimed to explore the illness experience of individuals of 0Æ3mgkg–1 day–1 with the total daily dose rounded off to with acne in relation to their treatment-seeking and adherence the nearest multiple of 10. Azithromycin was given at a dose behaviour. Thirty-eight patients attending a specialist acne cli- of 500 mg day–1 in 3 consecutive days every 2 weeks. The nic with a confirmed diagnosis of acne vulgaris took part in response to therapy in terms of percentage decrease in lesions semi-structured interviews. Twenty-four were aged 16–20 was assessed by the patients themselves at monthly intervals. years and 14 aged 30 years and above. Employing Interpretat- Side-effects, if any, were noted at every follow-up visit. Treat- ive Phenomenological Analysis the data were analysed for ment was continued until complete clearance of lesions. Those emergent themes. Four superordinate themes emerged: (i) patients who took regular treatment for at least 1 month were impact on day-to-day life and on the self, (ii) search for rem- included in final data analysis. Results: Of 38 patients inclu- edies, (iii) experience of health care and (iv) helping others. ded, 33 qualified for final data analysis. Twenty were male and Acne had an effect upon many daily activities as participants 13 were female with mean age of 21 years (range 15–36 strove to conceal their acne lesions from others. A wide vari- years). The mean age at onset was 17 years (range 10–32 ety of self help methods were used to try and treat and con- years) with the mean duration of illness being 4 years (range trol symptoms before seeking medical assistance. Factors 1–12 years). Twenty-six patients had Grade 3 and seven involved in the delay in seeking treatment were a lack of patients had Grade 4 acne. The mean treatment duration was knowledge that acne could be treated medically, a belief that 16 weeks (range 4–39 weeks). Twenty-one patients had 100% acne was a condition which everyone grows out of, and a response, eight and two patients each had 75–99%, 50–74% belief that acne was considered trivial compared with other and <50% response respectively. The total dose of isotreti- disorders. For the majority of both males and females it was noin per patient was 40 mg kg–1. There was no significant dif- the worsening of their appearance which prompted treatment ference in cumulative dose of isotretinoin between patients from GPs as they considered acne unsightly to themselves and with Grade 3 (38 mg kg–1) and Grade 4 (41 mg kg–1) acne. others. However, many participants perceived their GPs as not The mean follow-up period was 12 weeks (range 4–41 taking their disorder seriously. Participants had experienced weeks). Relapse occurred in only one patient, who initially prolonged courses of various medications before being had Grade 4 acne, received isotretinoin for 11 weeks and had referred to the specialist acne clinic. The majority of partici- a relapse after 8 weeks. Side-effects were seen in 27 patients, pants had persisted with these acne treatments despite side- the commonest being cheilitis (26 of 27). Others in decreasing effects. Of the minority who had stopped their medication this order of frequency were xerosis (7), facial erythema (6), apht- was due to side-effects such as dry and peeling skin which hae (2) and asteatotic eczema (1). None had a lipidogram they considered worse than the appearance of acne. Older

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 23 24 Bristol Cup Posters participants engaged in giving advice to others about acne, Inter-rater agreement on the ALC and GASS, using digital ima- whilst all believed more information needs to be disseminated ges, is consistent with in-person agreement rates reported in to prevent others from suffering as they had. This study sug- the literature. This indicates that remote management of acne gests the decision-making process to seek treatment and may be a practical management strategy for dermatologists. adhere to the treatment regime involves physical, psychologic- al and social factors. Attention to these factors will increase referral rates and adherence with medication. P-4 Conflict of interest: This study was funded by Stiefel Laborat- The use of isotretinoin – a regional audit in ories (U.K.) Ltd. Merseyside. Do proformas improve our documentation for patients on isotretinoin? K. Panting, N. Goyal, T. Sinha,* A. Memon,* P-3 G. Wong and J. Ellison Evaluation of digital images to assess clinical Broadgreen Hospital, Liverpool, Merseyside, U.K.; *Ormskirk District General progress in patients with acne Hospital, Ormskirk, Lancashire, U.K.; and Whiston Hospital, Prescot, A. Watson, H. Bergman, S-J. Seo, K. Tsai and Merseyside, U.K. J. Kvedar An audit into the regional use of isotretinoin in Merseyside Center for Connected Health, Harvard Medical School, Department of Derma- was carried out for all dispensed prescriptions in six hospitals tology, Massachusetts General Hospital, Boston, MA, U.S.A. between January and March 2006. The main aims of the audit Technology offers new ways to deliver care to dermatology were to assess adherence to the BAD guidelines (particularly patients. Digital images and clinical information can now be in terms of pretreatment counselling, investigation and monit- transferred from patient to clinician over the Internet allowing oring), to evaluate the use of the Pregnancy Prevention Pro- asynchronous, remote assessment. Acne patients are well-sui- gramme (PPP) in all females of child bearing age, to highlight ted to this approach as they are generally technologically able, any pregnancies, and to identify the incidence of any psychi- require ongoing follow-up but have a non malignant condi- atric symptoms. A proforma was completed for all available tion. Our study aimed to determine the ability of physicians case sheets and data analysed using Microsoft Access. A total to assess acne severity using such images, as this has not been of 246 patients were identified, of whom 58% patients were validated. Twenty subjects were recruited from an ongoing male and 42% female. The indication for isotretinoin in 94% clinical study into mild to moderate facial acne. Each patient of cases was for acne (with at least 82% of these documented had five study visits, at 6 weekly intervals. At each visit, sets as moderate to severe acne). As a region we are adhering to of three images (front and both sides) were obtained. Half of the BAD guidelines for pretreatment liver function and lipid the subjects were asked to take their own images, following a profile in over 97% patients. Pretreatment pregnancy testing is brief training session. The remaining 10 subjects had all ima- being performed in at least 93% of females of child bearing ges taken by study staff. In total 300 images were included in age and documenting monthly pregnancy testing throughout this analysis. All images were assessed by two dermatologists treatment in at least 79% females. We have also shown that using four validated acne assessment measures: the Acne isotretinoin is being prescribed in a safe and appropriate fash- Lesion Count (ALC); the Leeds Acne Assessment Scale; the ion. There were no pregnancies or reports of psychiatric dis- Global Assessment Severity Score (GASS); and a Forced Choice turbance amongst our cohort of patients. There was, however, evaluation. In addition, a nonmedical photographer rated the mixed performance across the region in terms of the docu- quality of the images using the Q-index. All assessors were mentation of pretreatment discussion of side-effects, previous blinded to the time-point of the image and to who had taken psychiatric history, pretreatment pregnancy testing and fol- the image. The mean age of participants was 26 years old (SD low-up discussion. One hospital had adopted a proforma for 5Æ84, range 16–40) and the majority (18) were female. The use in all patients on isotretinoin, resulting in significantly median ALC was 15 (IQR 8, 26Æ2), the median Leeds score better documentation compared with the region as a whole. was 0Æ5 (IQR 0Æ1, 0Æ8) and the median GASS was 1 (IQR 1, For example, 100% of patients using the proforma had writ- 2). Of the 300 images included in the study 10% taken by ten documentation of discussion of specific side-effects com- patients and 4% taken by study staff did not allow for any pared with less than 50% for specific side-effects amongst assessment of acne severity. The quality of photographs taken some hospitals using case sheet documentation. Of these the by study staff was significantly higher than those taken by risk of teratogenicity, depression or mood change, abnormal patients (P =0Æ003). Partial inter-rater agreement for the ALC lipid and liver function and dry skin and mucous membranes was 0Æ61 with absolute agreement of 0Æ54 for the GASS and were the most frequently recorded. The Pregnancy Prevention 0Æ38 for the Leeds Score. Interestingly, the quality of image Programme was used in 69% of females of child bearing age. did not appear to be related to the inter-rater agreement for However this was only implemented in some trusts during the ALC (r2 =0Æ03) or the GASS (r2 =–0Æ08). The majority our period of study; therefore the results may not truly repre- of digital images allowed an assessment of acne severity to be sent current practice. From a clinical governance and medico- made. Patients appear able to self-image although their images legal perspective thorough documentation of any discussion is may be of lower quality than those obtained in clinic. of vital importance. Our audit highlights the possible

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 25 significant improvement in documentation when using a pro- possible treatment and guiding them towards patient support forma and we have therefore recommended the implementa- groups if desired. tion of a proforma throughout the region. We plan to re- audit to reassess the quality of documentation, and hence complete the audit cycle. A copy of the proposed proforma P-6 will be included on our poster. A novel perspective on the significance of ferritin in postmenopausal hair loss? I. Ali, R.P.R. Dawber and F.T. Wojnarowska Oxford Churchill Hospital, Oxford, U.K. P-5 The aetiological role of iron deficiency in female hair loss is Survey on patients with polycystic ovarian syndrome an area of controversy. Iron deficiency has been associated (PCOS): what matters? M. Mehmi, P. Balasubramaniam, A. Abdullah and with both chronic telogen effluvium and female pattern hair S.W. Lanigan loss. Postmenopausal women form a unique group to study Birmingham Skin Centre, Birmingham, U.K. the relationship between iron stores and hair loss when there Patients undergoing laser epilation for polycystic ovarian syn- is lack of menstrual iron loss. This study aims to identify the drome (PCOS) suffer significant psychological morbidity as in- relationship between serum ferritin and hair loss in postmeno- dicated by quality of life (QOL) assessments. (Clayton WJ, pausal women. A detailed scalp examination by a single obser- Lipton M, Elford J et al. A randomized controlled trial of laser ver was given to 198 postmenopausal women; they were treatment among hirsute women with polycystic ovary syn- allocated a grade between 1 and 6 using a photographic scale drome. Br J Dermatol; 2005 152: 986–92). A departmental survey for both diffuse generalized and female (Lud- was conducted to explore patient perceptions about PCOS. A wig pattern). Subjects with thyroid disease were excluded. QOL questionnaire was filled in by 133 patients with PCOS Serum was collected under standard conditions for analysis of attending our laser clinic over a 3-month period for hirsutism serum ferritin. Because ferritin levels rise in systemic illness, treatment. Survey domains were derived from previously valid- parallel measurement of C-reactive protein (CRP) was concur- ated PCOS QOL questionnaires (Cronin L, Guyatt G, Griffith L et rently undertaken. Spearman rank correlation was applied to al. Development of a health-related quality-of-life questionnaire analyse the relationship between hair loss pattern and ferritin. (PCOSQ) for women with polycystic ovary syndrome (PCOS). No hair loss was shown by 134 women, 38 had female pat- J Clin Endocrinol Metab 1998; 83: 1976–87). One hundred and tern hair loss and 26 had diffuse generalized hair loss. Mean twenty-five of the 133 patients were bothered by hirsutism, 58 ferritin values were lowest in women with diffuse hair loss –1 –1 by weight gain, 31 by irregular menses, 22 by reduced fertility (47 ± 23 lgL ) compared with 65 ± 54 lgL for those and six by acne vulgaris (75 patients selected more than one with no hair loss. Interestingly, females with female pattern symptom). Confidence in personal relationships was impaired hair loss had the highest mean value for serum ferritin (97 ± –1 in 93 cases, in career and education in 46 patients, and in sports 104 lgL ). There was positive correlation between degree of and leisure activities in 38 instances. Only 10 patients stated female pattern hair loss and ferritin (P <0Æ05). Women with PCOS had no effect on their confidence. The most popular diffuse generalized hair loss had lower levels of ferritin but methods of hair removal prior to laser treatment were shaving this was not statistically significant. This study has found and waxing. Sixty-four patients were removing hair on a daily raised serum ferritin levels in postmenopausal women with basis, nine were removing hair a few times a week and the female pattern hair loss. Iron stores increase after the meno- remaining 60 patients were engaged in hair removal less fre- pause. Recent studies have shown raised ferritin levels to act quently. Although the face and neck were the most common as a marker of cardiovascular disease risk and metabolic syn- sites of hair removal, 52 patients also had to remove hair from drome. The results of this study suggest an alternative aetiol- the trunk and limbs. No information about PCOS had been ogy to iron deficiency in female pattern hair loss. Further received by 32 patients, 60 had downloaded information from work is required to identify the significance of postmenopau- the internet, 81 were given information by doctors, 16 by sal hair loss and raised ferritin as a marker of systemic disease. friends and family, and 15 obtained information from other sources such as magazines, books and medical journals. Only 32 patients were aware of a support group (support groups men- P-7 tioned by patients included Verity and the Silver Lining Infertil- Establishing rational investigations and appropriate ity support group). Further PCOS information was requested by treatments of hirsutism: an audit 107 patients. This survey illustrates that the most significant E. West, K. Mukkanna* and P. Yesudian symptom in PCOS patients attending our clinic is hirsutism. We Royal Liverpool and Broadgreen University Hospital Trust, Liverpool, Mersey- need to appreciate that hair removal can be a regular, time con- side, U.K.; *Southport and Ormskirk Hospital, Southport, Merseyside, U.K. suming, painful process involving large body surface areas. Der- and Countess of Chester Hospital, Chester, Cheshire, U.K. matologists should acknowledge the psychological morbidity Women with hirsutism frequently attend dermatology outpa- associated with PCOS. Holistic management includes providing tients complaining of its undesirable appearance and their patients with appropriate PCOS literature, delivering the best psychological distress. There are no recognized national or

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 26 Bristol Cup Posters international guidelines on hirsutism management, and there the keratin 5 or 14 genes (KRT5, KRT14). In contrast to are no published audits in the current literature. A retrospect- patients with other types of epidermolysis bullosa who ive case-note review over a 5-year period of patients with deteriorate at higher temperatures, those with DM-EBS hirsutism was undertaken in four hospital trusts. The history, sometimes report less blistering when they are pyrexial. We clinical features, investigations, management and psychological have documented this phenomenon, and demonstrated the impact were ascertained from the case-notes. Eighty-nine time relationship to temperature change. KH, a boy aged 2 patients were identified; age at presentation ranged from 14 years, has classical DM-EBS due to a de novo heterozygous to 65 years. The median symptom duration was 10 years arginine to histidine substitution at codon 125 (p.R125H) (range: 6 months–30 years). The menstrual history was recor- in KRT14. His parents, who always burst all his blisters ded in 81% of patients (33% had irregular cycles). The face every day to prevent them from spreading, noticed that he was the most frequently affected area with often more than developed fewer blisters when he had a high temperature. one cosmetic unit being affected (usually chin and upper lip). In order to study this phenomenon they agreed to keep a A pre-existing diagnosis of PCOS was found in 27%. Precon- daily record of number of blisters burst, and axillary tem- sultation physical treatments included: shaving (38%), pluck- perature using a digital thermometer, between August and ing (22%), waxing (18%), electrolysis (16%), bleaching December 2007. During the 107-day period of observation (15%), depilatory cream (13%), laser (12%) and topical eflor- he had three episodes of fever, defined as temperature nithine (6%). Preconsultation systemic treatments comprised: >36.9 oC, lasting 1, 2 and 5 days. Excluding the febrile co-cyprindiol (33%), metformin (11%), spironolactone (3%) episodes, and one week after each febrile episode, his med- and Yasmin (Schering Health, Burgess Hill, U.K.) (1%). Mul- ian baseline temperature was 36Æ1C (range 35Æ7–36.7) and tiple methods were frequently used. Psychological distress was the median blister count was 19 (range 13–31). On the recorded in 33% of patients and ranged from self-conscious- eight days of pyrexia, his median temperature was 37Æ9C ness to suicidal thoughts. Blood investigations were performed (range 37Æ0–38.5) and his median blister count was 5 in 68 patients (average four per patient), typically serum tes- (range 3–10). The temporal association of pyrexia and fall tosterone, FSH/LH and other endocrine tests. Most investiga- in blister count was highly significant (P <0Æ001). The fall tions were within the normal range (11% abnormal). Ovarian in blister count occurred on the first day of pyrexia, but ultrasound was performed in 26 patients. Seven new diagnoses the blister count remained low for several days after his of PCOS, two large ovarian cysts requiring a gynaecology temperature returned to normal. During the one-week peri- opinion, and one case of Cushing’s disease were identified. ods following febrile episodes his median temperature was Management was influenced by availability on the NHS: top- 36Æ0C (range 35Æ8–36Æ4) and the median blister counts on ical eflornithine and electrolysis were prescribed in 60% and each of the seven successive days following the febrile epi- 44% respectively. Funding limited the use of lasers. Systemic sodes were 9, 12, 13, 16, 18, 17, 17 showing a gradual medical treatment was prescribed for 13%. A precise history resumption of blistering. We believe this is the first attempt (especially the sudden onset of hirsutism, menstrual irregular- to quantify the reduced blistering reported with fever in ities and signs of virilisation) is more important than routine DM-EBS. Intermediate filament assembly is known to show investigations to establish any sinister cause for hirsutism. Hir- temperature dependence and EBS-mutant human keratins sute women are most likely to have PCOS, and imaging tech- show greater tendency to aggregate on heat shock (Morley niques to confirm this would be the standard management. SM, D’Alessandro M, Sexton C et al. Generation and charac- The single most important laboratory test is free testosterone, terization of epidermolysis bullosa simplex cell lines: scratch which if high can lead to more specific investigations. Feed- assays show faster migration with disruptive keratin muta- back from treated patients suggested that laser was the most tions. Br J Dermatol 2003; 149: 46–58). Further analysis of effective therapy. This audit has highlighted that treatment for the mechanism underlying the reduced assembly efficiency hirsutism is overall unsatisfactory unless epilation lasers are of mutant keratins may help us to understand why blister- available. ing in DM-EBS improves with pyrexia, and perhaps lead to new therapeutic interventions.

P-8 Blistering in Dowling–Meara epidermolysis bullosa P-9 simplex improves with pyrexia: a clinical study A scoring system for epidermolysis bullosa A. Wong, N. Hadley, M. Gulbransen,* B. Lane* and A. Wong, R. Ward, N. Hadley, D. James, C. Moss E. McAndrew, S. Richards, A.H.M. Heagerty* and Birmingham Children’s Hospital, Birmingham, U.K. and *University of C. Moss Dundee, Dundee, U.K. Birmingham Children’s Hospital, Birmingham, U.K. and *Solihull Hospital, Dowling–Meara epidermolysis bullosa simplex (DM-EBS) is a Birmingham, U.K. congenital skin disorder characterized by herpetiform clus- Epidermolysis bullosa (EB) is a diverse group of hereditary ters of intra-epidermal blisters, nail dystrophy and palmo- mechanobullous disorders encompassing three major forms: plantar keratoderma. It is caused by dominant mutations in simplex, junctional and dystrophic. There is currently no

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 27 system to quantify the severity of EB other than a subjective of the diagnosis of pemphigus foliaceus was made by both assessment as mild or severe. Objective scoring systems have routine histopathology and immunofluorescence. Various proved useful in other chronic skin disorders, such as treatments were employed including systemic steroids (oral SCORAD for eczema and PASI for psoriasis. We therefore prednisolone and latterly pulsed methylprednisolone), dap- attempted to devise a scoring system for EB. From discussion sone, azathioprine, intravenous immunoglobulins and nicotin- with EB professionals, we established the following require- amide. He is currently controlled on systemic prednisolone ments for an EB scoring system: it should cover all types of plus azathioprine. During the course of his disease currently EB, giving lower values for milder forms, and it should be present for 4 years, his exacerbations have occurred after con- simple, practical, reproducible between different observers and junctivitis on four occasions. PF is extremely rare in children sufficiently sensitive to pick up changes with age or treatment. outside endemic regions, such as Brazil and Columbia, where We initially identified four significant attributes: area of skin the epidemiology is highly suggestive of an autoimmune dis- damage, mucosal involvement, scarring to hands and nail loss. order triggered by an infectious insect-borne agent. Our case After trying out different numerical weightings, using virtual is of an extremely refractory PF in a young child from the patients with different types of EB, a system giving 50% of U.K., where the trigger would appear to relate to a staphylo- the points to the area of damaged skin and 50% to the other coccal conjunctivitis, a syndrome not previously reported. The three attributes was found to generate scores which correlated only effective treatment for our young patient has been high- best with our subjective classification. We then piloted the dose prednisolone, administered as pulses of methylpredniso- system on our patients with EB and modified and refined it lone. The use of pulsed methylprednisolone has not previously accordingly. We found it necessary to use the rule of nines been reported as effective therapy for PF in childhood. This diagrams to estimate affected areas, and to subclassify the treatment clearly has potential complications, most worryingly mucosae into eyes, mouth, larynx and oesophagus. We growth retardation, opportunistic infections and osteoporosis. reduced the contribution of nails, adding skin cancers, chronic The only accepted treatment for osteoporosis in children is wounds, alopecia and nutritional state into the scoring system. pamidronate infusions which were administered to our patient The scoring system was tested on 60 paediatric and adult whilst he was receiving high-dose steroids. The use of bis- patients with EB. The scoring system reflected severity, giving phosphonates in a child requiring high-dose steroids for PF the lowest scores to Weber–Cockayne EB simplex and highest has also not been previously reported. The use of bisphospho- to recessive dystrophic EB (RDEB). Within the RDEB group, nate infusions in conjunction with high-dose steroids in chil- score correlated with age and showed greater variability dren is discussed. between than within families. The scores showed a clear dis- tinction between patients previously considered mild or severe and highlighted families with unusually mild or severe pheno- P-11 types. Inter-observer variability appeared acceptable. Severity is Linear IgA disease of children (chronic bullous an important component of the phenotype of EB. Correlating disease of childhood): an update severity with genotype will help us to counsel parents of R. Clayton and F.T. Wojnarowska infants with EB. Furthermore, quantifying the severity of EB Churchill Hospital, Oxford, U.K. will be useful for clinical monitoring, measuring the effects of Linear IgA disease (childhood LAD) is an acquired blistering interventions, and estimating resource needs. We believe this disease with involvement of the skin and mucous membranes. scoring system will be useful in managing patients with EB The aim of our study was to identify the cohort of children and developing evidence for interventions. We plan to test this with LAD and describe the clinical and immunopathological scoring system with patients attending other EB clinics in the features. We performed a retrospective case-note review of all U.K. children referred, or whose serum was referred, to a teaching hospital with an interest in this disease. We identified 30 chil- dren under the age of 14 years at the age of presentation, all P-10 of whom had linear IgA on direct or indirect immunofluores- Juvenile pemphigus foliaceus cence (IF). The clinical features noted ranged from localized C. Williams, T. Clayton, M. O’Connell,* S. Clark and disease that was managed with topical steroids to widespread A. Layton* blistering and mucosal involvement that required systemic Leeds General Infirmary, Leeds, U.K. and *Harrogate and District Foundation treatment. Known triggers in six patients included immunisa- Trust, Harrogate, U.K. tion (1), infection (4) and medication (1). Genital involve- Pemphigus foliaceus is a rare disease in childhood. We report ment was documented in 18, six had ocular and four had oral a 5-year-old boy with pemphigus foliaceus (PF) triggered by involvement. Direct IF was positive in 19 patients with linear recurrent conjunctival infections. We believe this to be the basement membrane IgA, and four also had IgG. Indirect IF first case reporting this association. Our patient presented after on the serum was consistent with childhood LAD; in all 28 conjunctivitis with an arcuate erythematous scaly rash in a had circulating IgA basement membrane zone antibodies, two seborrhoeic type of distribution. He was otherwise healthy were also positive for IgG, and two negative but with IgA on and there was no family history of skin disease. Confirmation direct IF. Immunoblotting was performed on the serum of 18

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 28 Bristol Cup Posters patients. Nine patients had multiple antigens identified have progressed: no vesicles were seen, but she had developed (Table 1). Tissue typing for HLA class II antigens was carried multiple, discrete, hyperpigmented, greasy, verrucous papules out in 13 patients, 10 of whom had positive results (Table 2), over the dorsal aspect of her left forearm, hand and fingers. and six of these showed the classical HLA DR3 of this disease. Histology of a showed ulceration with surface crust Treatment varied with the severity of the disease. One patient and inflammation. Multinucleate keratinocytes with opaque was managed with topical steroids alone, but the others nuclei and margination of chromatin were identified within needed systemic therapy, mainly in the form of dapsone or necrotic debris at the base of the , consistent with a her- sulphamethoxypyridazine, prednisolone, or erythromycin. The pes virus infection. Varicella zoster DNA was detected via tis- prognosis was good; only two persisted into adult life, both sue polymerase chain reaction analysis confirming the being intermittently active, although one has remitted. This diagnosis of atypical cutaneous varicella zoster. She was review identifies the clinical and immunopathological features recommenced on high-dose oral valaciclovir (1 g t.d.s.) for 4 seen in this group. We have again found that genital involve- weeks which resulted in complete resolution of the lesions. ment is common; and scarring was not documented. This is With a persistently low CD4 lymphocyte count, she continued the largest series reported to date, and the only one in which on a prophylactic dose of valaciclovir (500 mg b.d.) and has macrolide antibiotics have been used successfully to control remained in remission. Atypical hyperkeratotic papules are an the disease. extremely rare cutaneous manifestation of VZV infection which have not previously been reported following stem cell Table 1. Antigens isolated by immunoblotting transplantation. Patients require prolonged high-dose antiviral treatment indicating that the pathogenesis is not necessarily BP180/97 BP230 LAD285 Collagen VII due to viral resistance. There is a reduction in virus-mediated No. of patients 15/3 8 6 1 keratinocyte lysis which suggests the virus may not be fully permissive within these cells, thereby facilitating their chronic nature. This case highlights the importance of recognising that Table 2. Tissue typing for HLA class II antigens immunocompromised individuals with very low CD4 lympho- cyte counts may present with atypical VZV infection and a B1 B8 DR2 DR3 DR4 DR7 DR13 DR15 DW3 high index of suspicion should be maintained when evaluating No. of patients 1 2 1 6111 1 1 these patients.

P-12 P-13 Atypical hyperkeratotic varicella zoster infection Type 1 (acquired) porphyria cutanea tarda occurring following stem cell transplantation in a father and son S. Clements, R. Carr* and N.B. O’Donoghue A. MacDonald and S.C. Holmes St John’s Institute of Dermatology, London, U.K. and *Guy’s and Glasgow University, Glasgow, U.K. St Thomas’ Hospitals NHS Trust, London, U.K. Porphyria cutanea tarda (PCT) is the most common of the Chronic cutaneous varicella-zoster virus (VZV) infection is as- porphyrias. The condition is due to a deficiency in sociated with prolonged suppression of cell-mediated immun- uroporphyrinogen decarboxylase (URO-D), an enzyme ity and loss of VZV-specific memory T cells. Hyperkeratotic involved in haem biosynthesis. Most cases are acquired VZV infection is an unusual entity that has been described in a (Type 1) but 10–20 % of cases are familial (Type 2). The small number of HIV positive patients and in several organ acquired form of PCT is associated with alcohol excess, transplant recipient patients. A 42-year-old Jamaican female hepatitis C virus (HCV) infection, human immunodeficiency with a 4-year history of adult T-cell lymphocytic leukaemia virus (HIV) infection, haemochromatosis and oestrogen and associated positive serology for HTLV-1 exposure, therapy. Classical clinical features include skin fragility, blis- received a BEAM Campath autograft, a highly immunosuppres- ters, erosions, scarring and milia occurring on photoexposed sive stem cell transplant. Twelve months later she remained sites. Atypical features such as dermatitis have only rarely pancytopenic with a low CD4 lymphocyte count of 40 been described (El-Mofty AM. Cutaneous porphyria in Egyp- cells mm–3. She was taking a regular prophylactic dose of aci- tians. Br J Dermatol 1964; 76: 268–77). We present a father clovir (200 mg q.d.s.) when she developed a small cluster of and son, both with the acquired form of the condition but erythematous vesicles on her left forearm consistent with her- each with hereditary haemochromatosis. The diagnosis of pes zoster virus reactivation. Despite increasing the dose of PCT was confirmed in each case by elevated urinary and oral aciclovir to 800 mg · 5 day–1 for 7 days, the eruption faecal porphyrin levels, plasma porphyrin screen and photo- extended to involve her left hand, so she was given high-dose testing. Each patient proved heterozygous for the Cys 282 oral valaciclovir (1 g t.d.s.) for 7 days until the blistering Tyr (C282Y) mutation of the haemochromatosis (HFE) gene. resolved. Her treatment was then changed to valaciclovir at a Both patients have exhibited atypical dermatitic features of prophylactic dose of 500 mg b.d. Six weeks later, despite PCT, and in one case this was the presenting feature. Both remaining on a prophylactic dose, the eruption was noted to patients responded well to venesection.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 29

by acral bullae in infancy and early childhood, generalized P-14 Propantheline bromide in the treatment of Hailey- progressive poikiloderma and diffuse cutaneous atrophy. It is Hailey disease: a case report generally agreed that the Kindler’s syndrome is a distinct clin- C. Roberts and R. Murphy ical condition. The patients all exhibit acral blistering from Queen’s Medical Centre, Nottingham, U.K. birth, and light sensitivity, both of which diminish with age Hailey–Hailey disease is an acantholytic dermatosis, with auto- from late infancy. They have poikiloderma appearing in early somal dominant inheritance, caused by a mutation in the infancy, which persists into early adulthood. This commences ATP2C1 gene on chromosome 3q21. Otherwise known as in sun exposed areas progressing to nonexposed areas. Many benign familial pemphigus, it usually presents in the third or patients exhibit mucosal damage. All the patients showed blis- fourth decades, and its clinical course tends to be mild but ter formation after trauma with cleavage along three distinct chronic. Clinical features include vesicles, erosions, cracks and dermal planes. We describe a sister and brother with Kindler’s fissures, mainly affecting the neck, axillae and groins as it is syndrome with the photosensitivity range to monochrome exacerbated by friction. Itching and pain may be present, radiation in the 300–400 nm range for the older sibling. The although worsening pain may represent infection with bacteria, children are from a consanguineous marriage between Asian yeast or virus. There are longitudinal white parents; there are four other siblings. In the case of the older bands in the nails of some patients. Histology demonstrates epi- sister the diagnosis was delayed as permission for a biopsy dermal hyperplasia with suprabasal separation and widespread was not originally given, and the condition was felt clinically acantholysis. Treatment is unsatisfactory and no controlled to be a variant of epidermolysis bullosa (Kindler T. Congenital therapeutic trials have been published, but management gener- poikiloderma with traumatic bulla formation and progressive ally involves conservative measures such as loose clothing and cutaneous atrophy. Br J Dermatol 1954; 66: 104–11. Abstract). absorbent pads as well as topical steroid treatment. Antibiotics However with the presentation of the second affected child a may be required long-term. Injection of botulinum toxin type A review of the cases was made and a further skin biopsy was has been reported to induce local remission (Lapiere JC, Hirsh taken from the older sibling which confirmed the diagnosis A, Gordon KB et al. Botulinum toxin type A for the treatment of (Weary PE, Manley WF Jr, Graham GF. Hereditary acrokeratot- axillary Hailey–Hailey disease. Dermatol Surg 2000; 26: 371–4), ic poikiloderma. Arch Dermatol 1971; 103: 409–22). As the presumably because the irritation and friction that results from majority of cases of Kindler’s syndrome develop poikiloderma- sweating is reduced. Other treatments include phototherapy, tous changes predominantly on light exposed areas we per- ciclosporin, dapsone, methotrexate, thalidomide, topical vita- formed monochromatic phototesting to ultraviolet light within min D3 analogues, surgery and laser ablation. A forty-seven- the range 300–400 nm to try to establish the range of photo- year old woman had been suffering from severe, widespread sensitivity. Hailey–Hailey disease, with recurrent infections and hospital admissions. It is likely that her diabetes mellitus had contributed to her phenotype being more severe than that of her sister, who P-16 had no concomitant medical conditions. Her treatments inclu- Atypical clinical manifestations in a patient with ded emollients, potassium permanganate baths, prednisolone Ehlers–Danlos syndrome type VIB 1 mg daily, acitretin 10 mg on alternate days and trimethoprim S. T. Cheung and C. Moss 200 mg b.d. It was felt that sweating may be exacerbating her Birmingham Children’s Hospital, Birmingham, U.K. disease, and due to the generalized nature of her condition, Ehlers–Danlos syndrome (EDS) type VIB (kyphoscoliotic type) injection with botulinum toxin type A was not appropriate, so (OMIM 229200) is one of the few types of EDS for which the treatment with oral propantheline bromide was commenced. A cause is unknown. We report a patient with the characteristic maintenance dose of 30 mg q.d.s. has been effective in improv- clinical features of EDS type VI, and several manifestations not ing her overall condition and reducing the frequency of hospital previously described together in this condition. This girl was admissions. Propantheline bromide is an anticholinergic drug, born at full term from consanguineous Bangladeshi parents. At used in the treatment of hyperhidrosis. Its use is often limited birth, she had hypotonia with joint laxity, macrocephaly and by side-effects such as dry mouth and urinary symptoms; how- talipes equinovarus. At 1 year of age, a communicating hydro- ever, in this case it has been well tolerated. We propose that it cephalus with a Dandy–Walker malformation was detected may be useful in selected cases of Hailey–Hailey disease. and later treated with a ventriculoperitoneal shunt. Orthopae- dic problems included flexion contractures at the elbow joints, P-15 recurrent spontaneous joint dislocations and scoliosis. Other Kindler’s syndrome in Asian siblings and an dysmorphic features included a rather flat mid-face, long slen- assessment of the photosensitivity spectrum der and cylindrical fingers and toes, hyperextensible skin and K. London and A.L. Wright ‘cigarette paper’ atrophic scarring. Her palm and sole creases Bradford Teaching Hospitals, NHS Foundation Trust, Bradford, West York- had a fine diamond shaped pattern. She also had rocker bot- shire, U.K. tom feet with piezogenic pedal papules. Ocular abnormalities Kindler’s syndrome or hereditary bullous poikiloderma was included bilateral microcornea, myopia and blue sclerae. At first described by Theresa Kindler in 1954. It is characterized the age of 20 years, she underwent surgical correction of

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 30 Bristol Cup Posters glaucoma and spontaneous retinal detachment. A skin biopsy by cold exposure and minor trauma. Although he gained no showed thin collagen fibres with a mild increase in elastic benefit from cyproheptadine, he did find relief from colchi- fibres in the reticular dermis. Electron microscopy demonstra- cine. FCAS is a rare autosomal dominant autoimmune disease ted relatively spherical collagen fibres and a lack of cauli- presenting as recurrent episodes of an urticated rash, fever and flower-shaped fibres. There were normal levels of arthralgia which are usually triggered by cold exposure some hydroxylation of lysyl residues in her skin, and urine bio- hours beforehand. The duration is lifelong and it normally chemistry revealed a normal ratio of hydroxylated to unhy- develops in childhood. It is due to a mutation in the NLRP3 droxylated urinary pyridinolines, excluding lysyl hydroxylase (formerly CIAS1) gene on chromosome 1q44 (Shpall RL, Jeffes deficiency (EDS type VIA). Chromosomal analysis showed a EWB, Hoffman HM. A case of familial cold autoinflammatory normal female karyotype (46, XX). EDS type VIB is a clinically syndrome confirmed by the presence of a CIAS1 mutation. Let- heterogeneous group. This patient was previously reported as ter. Br J Dermatol 2004; 150: 1029–31). This encodes the pro- a possible new syndrome (Mckee SA, Barnicoat A., Fryer A et tein cryopyrin which is thought to play an integral role in al. Joint and skin laxity with Dandy-Walker malformation and stress-associated pathways and interleukin-1 induced inflamma- contractures: a distinct recessive syndrome? Clin Dysmorph tion. The syndrome must be differentiated from acquired cold 2001; 10: 177–80). However, the diagnosis of EDS type VIB urticaria and Muckle–Wells syndrome. FCAS does not respond later became apparent. Joint contractures and talipes equinova- to antihistamines. Treatment is dependent on severity of symp- rus have been described in EDS type VIB but not in association toms and may include oral steroids, anabolic steroids, nonste- with all her intracranial and ocular abnormalities (Kosho T, roidal anti-inflammatory drugs, colchicines or, more recently, Takahashi J, Ohashi H et al. Ehlers-Danlos syndrome type VIB interleukin-1 receptor antagonist therapy. We present this case with characteristic facies, decreased curvatures of the spinal to highlight this rare condition and the unusual triggers in our column, and joint contractures in two unrelated girls. Am J patient. Med Genet 2005; 138A: 282–7). Our case further illustrates the varied manifestations of this condition and that diagnostic clinical features may take years to manifest. P-18 A case of LEOPARD syndrome associated with clinical features of Noonan syndrome and multiple P-17 neurofibromata An unusual presentation of familial cold C. Gleeson, A. Gulati, M. Patton* and R.C.H. Yu autoinflammatory syndrome University College London Hospital, London, U.K. and *St George’s R. Barry and L.J. Cook University of London, London, U.K. St Mary’s Hospital, Portsmouth, U.K. LEOPARD syndrome is an autosomal dominant condition, A 32-year-old man presented with a history of recurrent rash, characterized by multiple lentigines, electrocardiographic con- arthralgia and fevers which started in childhood. He also had a duction abnormalities, ocular hypertelorism, pulmonary steno- four-year history of right eye pain and inflammation. The rash sis, abnormalities of genitalia, retardation of growth and was episodic and consisted of tender urticated 1–2 cm plaques deafness. LEOPARD syndrome shares many clinical features which lasted for 24 – 48 hours. The rash was triggered by cold with Noonan syndrome. Clinical overlap between Noonan exposure, tiredness and certain alcoholic drinks. It was usually syndrome and neurofibromatosis is also recognized; however, associated with moderate to severe arthralgia of the small joints co-existence of features of all three of these conditions in one of the hands and feet and occasional febrile episodes. There patient is uncommon. We present the case of a 60-year-old was a long standing history of mouth ulcers. There was a lady with multiple lentigines of the skin since early childhood strong family history of ‘rheumatism’ and a ‘tiredness rash’. and short stature, consistent with LEOPARD syndrome. She Investigations revealed normal full blood count, renal and liver also exhibits clinical features of Noonan syndrome, diagnosed profiles. Rheumatoid factor, complement levels and autoim- at the age of twelve, with a history of developmental delay in mune screen were normal as were immunoglobulin levels and childhood, dyslexia, low posterior hairline with coarse curly protein electrophoresis. Hepatitis, EBV, rubella and parvovirus hair and pulmonary stenosis. In addition, she has had several serology were negative. Direct antiglobulin and anti-CD3 tests cutaneous neurofibromata excised and recent radiological in- were negative. Cold agglutinins were not detected. There was vestigations suggest the possibility of widespread internal neu- mild elevation of erythrocyte sedimentation rate (31 mm in rofibromata. On clinical examination there were multiple the first h) and C-reactive protein (29 mg L–1). The initial lentigines on her trunk and limbs. Her height was below the diagnosis was urticarial and he was treated with anti- third centile. There was a soft ejection systolic murmur at the histamines with little effect. While on a subsequent holiday in left sternal edge. Histological analysis of a pigmented lesion the U.S.A., he developed meningitis and a bilateral lower limb showed a lentigo, whilst several cutaneous neurofibromata petechial rash. He was diagnosed with familial cold autoin- have been identified. Cardiac catheterization and angiography flammatory syndrome (FCAS). More recently, he developed have confirmed the presence of pulmonary stenosis. A com- swelling and a vasculitic rash of the lower limb extremities puted tomography scan identified multiple low density lesions along with severe arthralgia. These symptoms were triggered exiting each neural foramen throughout the spine, suggestive

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 31 of multiple retroperitoneal neurofibromata. Chromosomal an- cell forms which displayed frequent ring configurations of alysis in 1968 showed normal Barr bodies and a 46XX karyo- the nuclei. A degree of exocytosis was present though not type. Further cytogenetic studies in 1999 confirmed the prominent. Special stains showed paucity of elastic fibres in absence of mosaicism in the skin. Mutation analysis has identi- the papillary dermis and immunohistochemistry confirmed a fied a mutation in exon 12 of the PTPN11 gene (position dense T cell rich lymphocytic infiltrate with the multinucle- 1403, C fi T). Mutation analysis of the neurofibromin gene, ate cells staining positively with CD68 and MAC 387 mark- NF1, which causes neurofibromatosis type 1, is ongoing. The ers. A diagnosis of CTCL was made, with the presence of PTPN11 gene encodes a tyrosine phosphatase involved in signal giant cells suggesting a granulomatous variant and the clin- transduction pathways. Mutations in this gene occur in 40% ical appearance in keeping with that of granulomatous slack of patients with Noonan syndrome and in 90% of patients skin. This case represents an unusual cause of pruritus ani with LEOPARD syndrome, confirming that these disorders are and highlights the need for awareness of the granulomatous allelic variants The PTPN11 mutation identified in this case slack skin variant of CTCL with a view to initiation of occurs at a site frequently mutated in LEOPARD syndrome. appropriate investigations and follow-up. It also highlights This mutation may also account for the phenotypic features of the need to have a low threshold for biopsy in cases of Noonan syndrome seen in this case. However, mutations in persistent dermatological conditions in particular if resistant the neurofibromin gene have been reported in patients with to treatment. neurofibromatosis–Noonan syndrome. Ongoing mutation an- alysis will help assess if this lady’s multiple neurofibromata are the consequence of a distinct gene defect in the neurofi- P-20 bromin gene or the result of altered cell signalling pathways Two cases of pustular toxic epidermal necrolysis secondary to the mutation in PTPN11. J. Hague, M. Kaur,* S. Orpin, R. Lim,* A.H.M. Heagerty, R. Charles-Holmes and H.M. Lewis* South Warwickshire General Hospitals NHS Trust, West Midlands, U.K.; P-19 *University Hospital Birmingham NHS Trust, Birmingham, U.K. and Granulomatous slack skin manifesting as pruritus Heart of England NHS Trust, Birmingham, U.K. ani: a rare cause of a common condition Toxic epidermal necrolysis (TEN) is a life threatening D.A. Thompson, S. Harris* and A.G. Smith immune-mediated reaction, characterized by severe cutaneous Department of Dermatology, University Hospital of North Staffordshire, and mucosal blisters and erosions. It often presents with flu- Stoke-on-Trent, U.K. and *Department of Histopathology, Mid Staffordshire like symptoms, followed by a maculopapular, urticarial, General Hospital, Stafford, U.K. purpuric, or erythema multiforme-like eruption, which then Granulomatous slack skin is a rare variant of cutaneous evolves into blisters and sheet-like erosions. Presentation with T-cell lymphoma (CTCL), occurring in less than 1% of all pustules, however, is not well described in the English litera- CTCL cases (McFarlane V, Friedmann PS, Illidge TM. What’s ture, and may lead to a delayed diagnosis. We present two new in the management of cutaneous T-cell lymphoma? Clin unusual cases of TEN that initially presented with pustular Oncol (Roy Coll Radiol) 2005; 17: 174–84). We report a case lesions. Patient 1 is a 19-year-old woman who was pre- of this uncommon condition manifesting as pruritus ani. A scribed penicillin V for an upper respiratory tract infection. 73-year-old male patient was referred to the dermatology She had developed widespread erythema 24 hours later with department in March 2001 by the colorectal surgeons by soreness of her vulva, lips and eyes, associated with pyrexia whom he had been seen for rectal bleeding and discharge and malaise. The macular-papular erythematous eruption was secondary to haemorrhoids, which were treated by banding. studded with pustules on her face, trunk, upper arms, and He had an 18-month history of pruritus ani unresponsive thighs, and there were severe mucosal erosions. Over the to treatment with potassium permanganate soaks, topical next 4 days she developed widespread blisters and erosions steroids and oral sedating antihistamines at nights. His rele- involving 80% of her body surface area, with a positive Nik- vant past medical history was that of atopy, having had olsky sign. This was consistent with progression to TEN and both asthma and eczema. Examination revealed an erythe- was confirmed histologically. Patient 2 is a 49-year-old man matous, severely excoriated peri-anal plaque, with some with metastatic lung deposits of rhabdomyosarcoma. He had areas of superficial ulceration, and atrophy with wrinkling completed a 5-day course of intravenous levofloxacin and and mild laxity of the skin. Several areas of lichen simplex co-amoxiclav for a presumed chest infection 2 days earlier were observed to affect the limbs and these were treated and presented with erythema of his chest and flexures with with a moderately potent steroid ointment with good numerous pustules. Mucosal sites were unaffected. Biopsy response. Patch testing to the European Standard Series and showed intraepidermal and subcorneal pustules consistent the Medicament Series was performed in September 2005 with acute generalized exanthematous pustulosis (AGEP). By with no relevant positive results obtained. An elliptical skin the following day the patient had developed blisters on the biopsy taken from left buttock in May 2006 showed para- neck, abdomen and back and the Nikolsky sign was positive. keratosis, acanthosis and a dense mixed dermal infiltrate The clinical picture was now one of TEN and this was con- consisting of lymphocytes and macrophages including giant firmed histologically. Only 4 previous reports of TEN with

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 32 Bristol Cup Posters pustules exist, with only one case in the English literature and ciclosporin had a similar effect, but was complicated by where pustules occurred in conjunction with, rather than chronic renal failure. In 2005, thalidomide was commenced preceding the TEN erosions (Yang CH, Yang LJ, Jaing TH, as a treatment for nodular prurigo, pyoderma gangrenosum, Chan HL. Toxic epidermal necrolysis following combination and as a chemotherapeutic agent for multiple myeloma (in of methotrexate and trimethoprim-sulfamethoxazole. Int J Der- combination with cyclophosphamide and dexamethasone). At matol 2000; 39: 621–3). It is possible that pustules are a a dose of 50–100 mg daily and with close monitoring of the relatively frequent occurrence in TEN, but they may go underlying neuropathy a dramatic improvement in prurigo unrecognised or unreported, particularly if they are an early nodules was observed with full symptomatic control and flat- phenomenon. Recent work on the immunological mechanism tening of all skin lesions. Over a 12-month period no new of TEN and AGEP suggests that there is some similarity in areas of pyoderma developed; furthermore, existing areas of their pathogenesis (Lerch M, Pichler WJ. The immunological ulceration stabilized and began to regress. This is an unusual and clinical spectrum of delayed drug-induced . case of POEMS syndrome with severe resistant pathergic pyo- Curr Opin Allergy Clin Immunol 2004; 4: 411–19). derma gangrenosum and recalcitrant nodular prurigo which Dermatologists and other health care professionals need to be highlights the effectiveness of thalidomide in these sometimes aware of the varied presentations of potentially lethal drug unresponsive conditions. reactions.

P-22 P-21 Lipoedematous scalp Pathergic pyoderma gangrenosum associated with G. Wylie, C. Clark, C. Black* and G. Roditi nodular prurigo in a patient with POEMS syndrome Glasgow Royal Infirmary, Glasgow, U.K. and *Western Infirmary, Glasgow, treated with thalidomide U.K. N. Desai, J. Berens, A. Mehta,* C. Orteu* and Lipoedematous alopecia is an uncommon idiopathic cause of C.A. Holden scarring alopecia that occurs most frequently in black St Helier Hospital, Carshalton, U.K. and *Royal Free Hospital, London, U.K. women (Martin JM, Monteagudo C, Montesinos E et al. Li- POEMS syndrome is a multisystem disorder characterized by pedematous scalp and lipedematous alopecia: a clinical and plasma cell dyscrasia in association with polyneuropathy, histologic analysis of 3 cases. J Am Acad Dermatol 2005; 52: organomegaly, endocrinopathy and skin changes. Classical skin 152–6). The disorder is typically asymptomatic but scalp in- changes of the syndrome include , skin flammatory change and hyperlipidaemia (Bukhari I, Al Mul- thickening and sclerodermatous change, hypertrichosis and him F, Al Hoqail R. Hyperlipidemia and lipedematous scalp. multiple angiomata. Pyoderma gangrenosum is well documen- Ann Saudi Med 2004; 24: 484–5) have been reported. Hair ted in association with the underlying M-band. Pruritus and loss is associated with hypertrophy of the subcutis that can nodular prurigo have not been reported in isolation with be demonstrated by histology, ultrasound, computed tomog- POEMS, the aetiology in such patients is often multifactorial. raphy or magnetic resonance imaging (MRI). However, lip- We describe a 62-year-old woman with POEMS syndrome oedematous scalp (LS) has been recognized as a rare subset who had been under the joint care of dermatology and rheu- of this disorder where subcuticular hypertrophy occurs in matology departments for the past 18 years. Her systemic fea- the absence of hair loss. We report LS occurring in a white tures included widespread sclerodermatous skin change, woman who has dense scalp hair growth. The 56-year-old generalized hyperpigmentation, paraproteinaemia progressing woman was aware of a fluctuant sensation over the poster- to frank myeloma, diabetes, hypothyroidism, peripheral neur- ior aspect of her scalp for over 5 years. She was otherwise opathy and an associated erosive polyarthropathy. In addition, well with no previous trauma or surgery to the head or she had a 10-year history of recurrent ulceration of the lower scalp. She was not hyperlipidaemic. Palpation revealed a dif- limbs, histologically and clinically consistent with pyoderma fuse, soft, sponge-like texture and thickening over the scalp gangrenosum, and a 5-year history of generalized pruritic skin most evident at the vertex and parieto-occipital areas. The with extensive excoriated prurigo nodules densely involving scalp was otherwise normal and she had a dense covering the trunk and all four limbs. Despite optimizing biochemistry of scalp hair with no evidence of alopecia or dermatosis. In- profiles (iron, thyroid and renal) these nodules progressed cisional scalp biopsy demonstrated essentially normal scalp and were complicated by pathergic spread of new pyoderma- histology beyond expansion of the subcuticular adipose tous ulceration on the thighs and lower legs. Both pyoderma layer. Alcian blue mucin stains were negative. This was also gangrenosum and nodular prurigo were resistant to multiple confirmed with ultrasound and MRI and measurement of topical and systemic therapies including topical clobetasol a maximum scalp thickness of 15 mm (normal range propionate (Dermovate ; GlaxoSmithKline, Uxbridge, U.K.), 5–6 mm). Her condition remains unchanged on clinical topical tacrolimus, oral prednisolone, cyclophosphamide, az- review. LS is a rare acquired benign scalp disorder of athioprine, dapsone, hydroxyzine and intramuscular and intra- unknown aetiology that is best demonstrated with MRI ima- venous methylprednisolone. Two-monthly intravenous ging. Fortunately, alopecia is not a feature as therapeutic immunoglobulin provided partial improvement in the prurigo, interventions are limited.

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P-23 strates evidence of evolution of PNGD along this clinicopatho- Palisaded neutrophilic granulomatous dermatitis logical spectrum over the course of 7 years. associated with systemic lupus erythematosus presenting with the ‘burning rope’ sign A. Gulati, D. Paige, R. Cerio, M. Khorshid, P-24 M.M. Yaqoob,* C.M. Proby and C.A. Harwood Shiitake dermatitis: a case diagnosed after 16 Department of Dermatology and *Department of Renal Medicine and years! Transplantation, Barts and the London NHS Trust, London, U.K. S. Garg and S. Cockayne A 42-year-old woman with a history of systemic lupus erythe- Department of Dermatology, Royal Hallamshire Hospital, Sheffield, U.K. matosus (SLE) and lupus nephritis presented in 1999 with Shiitake dermatitis is a condition that commonly occurs in ori- sudden onset of an exquisitely tender red plaque in the right ental countries and has recently been reported in Europe. We axilla. Herpes zoster was diagnosed and she was treated with would like to report the case of a 45-year-old Swiss man with oral aciclovir and the plaque resolved over 2 weeks. Over the a 16-year history of an intermittent rash. He described a next 2 years she experienced six similar episodes affecting mildly pruritic rash mainly on his trunk, with an associated both right and left axillae, each associated with fever and mal- strange and warm sensation. The rash lasted approximately 1 aise and spontaneously settling over 2–3 weeks, leaving prom- week and was not helped by antihistamines. He denied any inent postinflammatory hyperpigmentation. Investigations in obvious precipitants. On examination, grouped linear weal- 2001 revealed an antinuclear antibody (ANA) titre of >1 : like lesions were observed, especially over the central back. 640, and positive anti-Ro, anti-RNP and dsDNA antibodies. Blood tests revealed an eosinophilia during the time the rash Histologically, a perivascular and interstitial inflammatory was present, although normal in between. All other tests were infiltrate with focal extravasation of red cells and nuclear dust normal. Further questioning revealed an interest in oriental was suggestive of a resolving vasculitic process. Strong linear food and cooking including shiitake mushrooms, and previous IgG staining of the basement membrane was seen on direct episodes occurred after visits to hotels and restaurants where immunofluorescence. The diagnosis was uncertain but the epi- shiitake mushrooms may have been consumed. The diagnosis sodes appeared to improve with the introduction of azathio- was confirmed with an oral challenge of half-cooked shiitake prine for lupus nephritis. However, she was re-referred in mushrooms, 36 h after which the patient developed the rash. 2006 having continued to experience intermittent flares which Shiitake dermatitis or toxicoderma is a condition that occurs had increased in frequency and severity and were now associ- after the ingestion of raw or half-cooked shiitake mushrooms ated with an intense burning sensation, fever, malaise and (Lentinus edodes). First described in 1977 by Nakamura, it is well arthralgia lasting up to 3 weeks. Bizarre, erythematous, hot, reported in Japan and China; however, it has recently been tender, linear and markedly indurated subcutaneous ‘cords’ reported in the U.K. The clinical features are linear groups of extended from anterior and posterior axillae across the lateral pruritic erythematous papules or flagellate dermatitis normally chest wall, for up to 30 cm, clinically suggestive of a severe up to 48 h after ingestion. This is a similar pattern to that of thrombophlebitis. ANA was >1 : 640 and dsDNA enzyme- bleomycin-induced flagellate dermatitis. Shiitake mushrooms linked immunosorbent assay was strongly positive at 96 IU can cause allergic reactions. However, it is suggested that shii- –1 mL (negative < 30). Histology revealed a deep dermal take dermatitis is caused by a toxic reaction to the polysaccha- mixed inflammatory infiltrate centred on hyalinized and ride lentinan. This is also responsible for the beneficial effects degenerate collagen bundles with prominent neutrophils and of shiitakes. The patient did not link his episodic eruption nuclear dust but no frank vasculitis, features consistent with with shiitake mushrooms himself due to the time lag of at SLE-associated palisaded neutrophilic granulomatous dermatitis least 1 day between ingestion and symptoms. Also, the mush- (PNGD). PNGD is a rare cutaneous manifestation of connective rooms did not always cause a reaction as the lentinan can be tissue disorders first described in 1965 (Dykman CJ, Galens destroyed with thorough cooking. The popularity of oriental GJ, Good AE. Linear subcutaneous bands in rheumatoid arthri- cuisine has increased over the years in Europe and this case tis. An unusual form of rheumatoid granuloma. Ann Intern Med highlights the importance of recognizing an unusual disease, 1965; 63: 134–40), usually in association with rheumatoid which may occur for many years before presentation. arthritis, and only occasionally reported with SLE. The original clinical descriptions were of indurated linear and arcuate cords or bands situated on the lateral aspect of the trunk, giving rise P-25 to the term ‘rope’ sign (although ‘burning rope’ sign would Amyloidoma presenting as a skin tumour be a more accurate description of our case). The clinical mani- A. MacDonald, C. Moyes, K. Blessing and S. Holmes festations of PNGD are now recognized to represent a spec- Glasgow University, Glasgow, U.K. trum ranging from papules, plaques, nodules and annular An amyloidoma (amyloid tumour) is a localized mass of lesions through to these subcutaneous cords. A histological amyloid deposition with characteristic histological features. continuum also exists, from leucocytoclastic vasculitis in early Although nodular amyloid deposits affecting the skin are well lesions through to palisaded granulomas, altered collagen and described, a mass of amyloid mimicking a neoplasm is rare. dermal fibrosis in the later stages. Our case uniquely demon- We describe a case of amyloidoma arising within an

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 34 Bristol Cup Posters abdominal scar and presenting as a solitary, firm, well-defined ography) have been used to visualize VMs in the bowel; tumour. Histological examination confirmed amyloid depos- however most are invasive and some have limited sensitivity. In ition, with pathognomonic apple-green birefringence seen our case there was neither gastrointestinal bleeding nor anaemia; under polarized light in sections stained with Sirius Red. Char- however, multiple small bowel VMs were clearly demonstrated. acteristic fibrils were also observed with electron microscopy. Capsule endoscopy may allow us to identify patients who are at Special stains identified AL-type amyloid, which is caused by risk of complications and ensure appropriate management. accumulation of immunoglobulin light chains. No evidence of systemic amyloidosis or a monoclonal gammopathy was found after investigation. Amyloidomas have been previously repor- P-27 ted as occurring within bone, soft tissue, breast, lung, brain Morgellons disease: fibre or fiction? and nasopharynx. This is the one of few reported cases occur- S. Ogden and I. Coulson ring within skin and the second case within a surgical scar. Burnley General Hospital, Burnley, U.K. A 45-year-old woman was referred to dermatology with a 3- month history of small fibres of varying colour emanating from P-26 her skin. She had brought to the consultation multiple micro- Capsule endoscopy: a useful investigation in blue scope slides with small coloured fibres mounted upon them that rubber bleb naevus syndrome she said had mainly arisen from underneath the skin of her E. Hiscutt, N. Rajan, S. Panter* and S. Natarajan palms. She specifically denied any concerns that there was any Sunderland Royal Hospital, Sunderland, U.K.; and *South Tyneside District parasitic infestation of the skin, although she did describe her Hospital, South Shields, U.K. condition as an ‘infection’ and bathed at least once daily in anti- Blue rubber bleb naevus syndrome (BRBNS) is a sporadic or septic. She had diagnosed her condition as Morgellons disease autosomal dominant syndrome comprising of venous malfor- and had brought information about the disease downloaded mations (VMs). The combination of cutaneous VMs with gastro- from the Internet. Examination was unremarkable; vitamin B12 intestinal VMs is typical and onset of gastrointestinal levels and Lyme serology were checked and were normal. She haemorrhage is often noted by the second decade. BRBNS may was reassured that there was no evidence of infection in her skin; be part of a spectrum of disease and a subclass of familial VMs, however, she declined referral to psychology or psychiatry servi- which linkage analysis has mapped to chromosome 9p (Nahm ces and did not wish to consider treatment with antipsychotic WK, Moise S, Eichenfield LF et al. Venous malformations in blue medication. Morgellons disease is considered by most dermatol- rubber bleb syndrome: variable onset of presentation. ogists to be synonymous with delusions of parasitosis a form of J Am Acad Dermatol 2004; 50: S101–6). A 43-year-old woman monosymptomatic hypochondriacal psychosis. However, these presented with multiple, asymptomatic skin lesions that had patients complain of fibres or granules emanating from the skin gradually appeared over 25 years, which recently concerned her rather than parasitic organisms. There may be an associated psy- as they were enlarging and becoming palpable. She described a chiatric condition such as psychosis, depression or anxiety. vascular mass, which had been present from birth in the peri- Patients report symptoms such as sensations of itching, burning neal region. A solitary lesion on the tongue had previously been or things crawling on the skin as well as the production of fibres treated with cryotherapy. There was no history of gastrointest- or granules from the skin. The Morgellons Research Foundation inal bleeding or relevant past medical history, and there were was formed in America in 2002 and is dedicated to ‘finding the no similar disorders in her family. Examination of the skin cause of an emerging infectious disease, which mimics revealed multiple purple-blue soft papules, none more than and lice’. The founder of the Morgellons Research Foundation 5 mm in diameter on the neck, face, trunk, soles of the feet and derived the name from a condition described by Sir Thomas tongue. They were nontender and compressible with slow refill; Browne in the 17th century: ‘that endemial distemper of chil- the surface was smooth. There was a larger bluish vascular mass dren in Languedoc, called the morgellons, wherein they critically involving the perineum, right gluteal fold and labium major. break out with harsh hairs on their backs’. Management of The haemoglobin concentration at presentation was 14Æ2gdL–1 patients with this condition is challenging. Patients require thor- and was consistently within normal limits. Excision biopsy of ough examination and basic investigations to rule out organic the skin confirmed dilated thin-walled blood vessels within the disease. It has been suggested that once organic disease has been dermis, with no glomus cells present. Small bowel VMs were discounted, the use of the term ‘Morgellons disease’ when com- demonstrated by capsule endoscopy, which involves ingestion municating with patients about their condition may be a more of a video capsule after bowel clearance. The capsule transmits successful way of establishing a rapport, compared with the use approximately 50 000 images of the bowel during its transit; of the more traditional term of ‘delusion’, which is usually resis- here the recording revealed multiple blue vascular nodules of ted. Treatment options include antipsychotic medications such as varying size, with normal overlying mucosa in the jejunum and olanzapine, pimozide and risperidone. This is, to our knowledge, ileum. VMs in the gastrointestinal tract can bleed resulting in the first reported British case of Morgellons disease. The case chronic anaemia and there have been reports of fatal bleeding highlights the ever-increasing role of the Internet in providing with BRBNS. Other complications include volvulus, intussuscep- patients with potentially misleading information that can encour- tion and bowel infarction. Several imaging methods (e.g. angi- age self-diagnosis and resistance to appropriate treatments.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 35

P-28 the students, we decided to pilot an online e-learning com- Kikuchi’s disease: a case of skin involvement ponent to the dermatology attachment to replace the existing associated with non-small-cell lung carcinoma lectures. In order to evaluate this programme, we designed R. Meys, K. Skwarski,* D. Kemmett and J. Goodlad anonymous open-ended questionnaires to be completed by Department of Dermatology,*Respiratory Medicine and Department of the students before and at the end of their attachment. The 10 Pathology, Lothian University Heath Division, Edinburgh, Midlothian, U.K. online lectures were the highlight of the e-learning compon- Kikuchi’s disease (KD), also known as Kikuchi–Fujimoto dis- ent and consisted of 20-min PowerPoint presentations with ease or histiocytic necrotizing lymphadenitis without granulo- background audio which had been specially formulated to fol- matosis, is a rare clinical entity first identified in Japan in low the aims and objectives of the streamlined dermatology 1972 (Kikuchi M. Lymphadenitis showing focal reticulum cell curriculum. At the end of each lecture, the students were hyperplasia with nuclear debris and phagocytes. Acta Haematol directed to attempt multiple choice questions (MCQs) pertain- Jpn 1972; 35: 379–80). Characteristically, KD presents as a ing to each topic. The MCQs could be attempted many times. necrotizing lymphadenitis associated with fever, leucopenia, A problem-based learning (PBL) task involved students under- and systemic symptoms such as malaise, loss of weight and going the dermatology rotation at the same time to communi- arthralgia. A skin rash is present in 16–40% of cases (Kuo TT. cate online to answer a specific question, which then Cutaneous manifestations of Kikuchi’s histiocytic necrotizing culminated in five 10-min presentations at the end of the lymphadenitis. Am J Surg Pathol 1990; 14: 872–6). Most cases block. One interactive session aimed to strengthen the stu- have been reported from South-East Asia and in younger dents’ understanding of melanoma, while another assessed patients. Numerous associations have been made, most com- their ability to distinguish between benign and malignant le- monly with systemic lupus erythematosus and viral infections, sions. Of the 352 responses received for the pre-attachment but the aetiology of KD remains unknown. Our case occurred questionnaires 89% of students expected the online lectures to in a 70-year-old man with metastatic non-small-cell lung can- be of good quality, useful and easily accessible. Ten per cent cer. He was treated with palliative chemotherapy, and within of students expressed their reservations. Of the 410 post- days developed tender, erythematous plaques and nodules attachment questionnaires received, 63% thought that the over the face and trunk. He also had an unusual distribution online lectures had been useful, whereas 25% felt that the of mediastinal nodal enlargement and severe periorbital online lectures met their expectations. Eleven per cent oedema mimicking superior vena cava obstruction. Other fea- expressed difficulties, mainly in accessing the lectures. The tures included fever, malaise, confusion, neutropenia and majority of the students felt that the PBL task had been a waste abnormal hepatic and renal function. The rash persisted for 21 of time. Five per cent felt that other specialities should follow days and then resolved spontaneously. Skin biopsy, performed the dermatology online format. The success of the online after the onset of the rash revealed a perivascular and periad- component can be attributed mainly to the online lectures. nexal lymphohistiocytic infiltrate associated with abundant The failure of the PBL task could be linked to the fact that it karyorrhectic nuclear debris. The histiocytes possessed crescen- was not a summative assessment and therefore the students tic or twisted nuclei with finely dispersed chromatin, and felt it was a waste of time. We feel that the lectures succeed- reacted positively with antibodies to myeloperoxidase as well ed, as they were short, mirrored the aims and objectives of as CD68. Superficially, the infiltrate was associated with mild the curriculum and were accessible to students at their con- dermal oedema and vacuolar change in basal keratinocytes. venience. These factors greatly helped in preparation for the This case of KD has a number of unusual features: onset age is end of block exams. Perhaps dermatology departments at the outer spectrum of previously reported cases, the pre- throughout the U.K. could implement the online lecture for- senting rash was florid compared with previously documented mat in order to enhance the learning experience for students. cases, and associated eye oedema is documented but infre- quent. Additionally, this case is notable in that it presented as a paraneoplastic phenomenon, which may have either been P-30 reactive to chemotherapeutic intervention or the tumour itself. Dermatology and junior doctors: an evaluation of education, perceptions and competencies W. Hussain, J. Hafiji,* A. Stanley and K. Khan P-29 The Dermatology Centre, Hope Hospital, Salford, Manchester, U.K.; Evaluation of a pilot online dermatology programme *Department of Dermatology, Addenbrooke’s Hospital, Cambridge, U.K.; for undergraduate medical students Department of Cardiovascular Sciences, University of Leicester, Leicester, J. Vyas, A. Salmoni, M. Gonzalez and A.Y. Finlay U.K. and Department of Cardiology, North East Wales NHS Trust, Cardiff University, Cardiff, Wales, U.K. Wrexham, U.K. The fourth year undergraduate students undergo a week’s Dermatology is now established as part of the core curriculum dermatology rotation as part of their 7-week block of module of most U.K. medical schools and is a recognized component of subspecialties. Traditionally, the students were given a ser- of the curriculum for trainees in general internal medicine. ies of didactic lectures in the first and seventh week of the We undertook a survey of junior doctors attending three con- Moss block. In order to enhance the learning experience for secutive preparatory courses for the Membership of the Royal

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 36 Bristol Cup Posters

College of Physicians (MRCP) Part II Clinical Examination sible. As a result of a paucity of undergraduate training, many (PACES – practical assessment of clinical examination skills) general practitioners (GPs) feel ill-equipped to manage derma- during 2006 in order to ascertain the extent of dermatology tology patients. In an attempt to identify training needs for experience at undergraduate and postgraduate levels. Respond- GPs, a local service evaluation was performed. Seventy GP ents were also asked to rate statements regarding their percep- practices were mailed with a needs analysis questionnaire. This tions of dermatology using a 10-point Likert scale (1, strongly embraced questions on the number of dermatology cases disagree; 10, strongly agree). An individual’s perceived com- being seen in the community with particular reference to petence in performing routine bedside dermatological investi- acne, eczema, psoriasis and/or skin cancer, perceived educa- gations was also assessed. A total of 118 questionnaires were tional needs required to manage these common conditions, evaluated (100% response rate). The majority of doctors were perception of what the local secondary care service actually in their third year of general medical training. Only 4% of provides, as well as figures on how many patients from each respondents expressed a desire to pursue dermatology as a GP practice benefited from these services. As confirmed from possible career option post-MRCP. One-quarter had undergone previous studies, 10–15% of a GP’s workload was felt to relate a clinical attachment in dermatology as a medical student to a dermatological problem. The referral rate for secondary ranging from 2 days to 2 months in duration. Twenty-four of care referral was stated to be 11% for eczema, 18% for psoria- 118 doctors had some experience in dermatology at either sis, 4% for acne and 17% for skin cancers. GPs were keen for preregistration house officer or senior house officer level. Sig- education and supervision in psoriasis and skin cancers par- nificantly, 84% wished for more dermatology exposure at ticularly and believed that they would benefit from more help undergraduate level and this increased to 90% at postgraduate in educating patients about these conditions. On questioning level. Despite the possibility of encountering a dermatological the GPs about their perception of secondary care services, it condition in their PACES examination, only 20% of doctors was clear that they were not fully aware of all of the facilities felt confident in their ability to describe a rash and only 8% available for their patients. The low referral rate for some con- felt confident in initiating basic therapy for a skin complaint. ditions and the failure to appreciate the scope available within Surprisingly, one-third of doctors had never taken a skin swab the secondary care service would suggest that there were sig- and 90% had never performed a skin scraping and were nificant educational needs to ensure GPs are informed about unsure how to do so. The majority of doctors, however, felt all the therapeutic and management opportunities for their that all junior doctors should be competent in these proced- patients. Practitioners also highlighted the need to have better ures. Over half expressed that if dermatological training was information to help them with patient education. This study coupled with internal medicine allowing dual accreditation, highlights the need for targeted community education to they would seriously consider it as a career option. Over 70% ensure that GPs are aware of all potential therapeutic dermato- agreed that dermatology allows a person to achieve a favour- logical options in both primary and secondary care in order to able work-life balance, although a significant proportion manage their patients’ problems to the highest standards. (37%) considered it a ‘soft’ option for those wishing to stay Conflicts of interest: This work was funded in part by Leo in hospital medicine. Most doctors felt that MRCP remained a Pharmaceuticals Ltd. necessary requirement for dermatologists. The many cutaneous manifestations of systemic disease and the use of potentially toxic systemic therapies were felt to be the most important P-32 reasons for possessing this qualification. Our findings suggest The impact of clinical teaching fellows upon that despite the inclusion of dermatology in medical curricula, undergraduate dermatology junior doctors still lack confidence in even the most basic K. Gordon, H. Al-Sader and C. Harland aspects of the speciality. Increased dermatology training can St Helier Hospital, Surrey, U.K. help address this problem and may correct the misconceptions Clinical teaching fellow (CTF) posts were introduced to the that many still have regarding the speciality. team of undergraduate clinical teachers 6 years ago. CTF posi- tions typically attract post-membership doctors keen to further their teaching and clinical skills prior to obtaining National P-31 Training Numbers. Their numbers are increasing and posts are GP training needs analysis being created in a variety of medical specialities. Our district M. O’Connell, R. Robinson, K. Baxter, L. Bottomley, general hospital (DGH) made the decision to appoint a derma- S. Das and A.M. Layton tology CTF in September 2004, to assist with the education of Department of Dermatology, Harrogate and District NHS Foundation Trust, undergraduate students from the nearby teaching hospital Harrogate, North Yorkshire, U.K. (TH). Third-year medical students spent 5 weeks attached to The government campaign to place ‘care closer to home’, the dermatology, ENT and ophthalmology firms at either the DGH introduction of payment by results, current financial con- or the TH. The latter group received teaching from the derma- straints for primary care trusts (PCTs) and the political thrust tology team. They did not receive teaching from a dedicated to introduce private initiatives has resulted in PCTs trying to CTF, unlike the DGH students. The CTF provided twice- reduce as many secondary care dermatology referrals as pos- weekly clinical teaching in the outpatient department, in

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 37 addition to opportunistic teaching and small group seminars. given, a questionnaire was sent to all students. This contained Students from both groups sat a summative exam at the end a mixture of Likert-style questions and areas for free text. of their attachment, under strict examination conditions. We Twelve out of 16 students returned the questionnaire. All 12 attempted to assess the impact of the dermatology CTF on the students chose ‘agree strongly’ with the comment ‘I find the undergraduate curriculum by evaluating the end-of-attachment one-to-one tutorials a useful learning experience’. Ten out of examination results. The examination results were retrospect- 12 students felt that the tutorials helped them to feel less ively obtained from the teaching hospital’s registry for the alone in their study. All students wanted more individual academic years 2003/2004 (i.e. before the introduction of the tutorials. Eleven out of 12 students found the discussion board CTF), 2004/2005 and 2005/2006. The students’ scores were a useful way of communicating with other students and the divided into two groups: ‘DGH group’ (approximately 45 stu- tutor. Eleven out of 12 students found the tutor’s response to dents each year) and ‘TH group’ (approximately 120 students questions was efficient. The Likert answers and free text com- each year). The average mark for the academic year was calcu- ments suggest that the individual tutorials are an important lated for each group. The average examination mark attained part of the programme. All the students want more tutorials by the DGH group in 2003/2004 was 70%, rising to 75% in and in order to accommodate this and an increasing number 2004/2005 after the introduction of the CTF. The average of students, it will be necessary to recruit additional tutors. mark in 2005/2006 was even higher at 83%. The TH group’s The popularity of the discussion board was surprising to us, mark from 2003/2004 was 71%. This rose to 75% in 2004/ as it is our experience that few of the students post messages 2005 and 77% in 2005/2006. Although the examination on the discussion board. This needs further evaluation with marks increased over the 3-year period in both groups, the online focus groups at a later date. The tutor’s response is per- DGH group increased their marks by 20%, compared to 9% in ceived as efficient at present and this must continue. Distance the TH group. The overall increase in examination marks learning is predicted to be used increasingly in undergraduate could be due to a number of factors, including small sample and postgraduate education. Student support is a key require- size, improved undergraduate dermatology teaching at both ment. Our experience is likely to be of interest to all derma- sites, or due to students conferring between examinations. tologists involved in teaching. However, what is noticeable is the marked improvement in the DGH group since the introduction of the dermatology CTF. We believe our small pilot study highlights the benefits P-34 of introducing a dedicated teacher to the speciality of derma- A survey on the management of neurofibromatosis tology. type 1 and awareness of national guidelines among dermatologists S.T. Cheung and C. Moss P-33 Birmingham Children’s Hospital, Birmingham, U.K. Evaluation of new methods designed to support Neurofibromatosis type 1 (NF1) is one of the commonest students on a distance learning course in autosomal dominant disorders. It affects mainly the skin and dermatology peripheral nervous system but can affect any body system. V. Hubbard and V.M.L. Jolliffe The U.K. Neurofibromatosis Association (NfA) has published Centre for Cutaneous Research, Queen Mary’s University London, London, consensus guidelines on the management of patients with NF1 U.K. recommending annual standardized clinical examination, We introduced a new online distance learning postgraduate unless there is a symptom or complication that needs more diploma in clinical dermatology for doctors outside the U.K. frequent review (NfA. Clinical Guidelines for the Management of Indi- in May 2006. Teaching is via WebCT, an educational soft- viduals with Neurofibromatosis 1. Kingston upon Thames: Neurofi- ware package that provides written material, clinical slides and bromatosis Association, 2003; Ferner RE, Huson SM, Thomas interactive formative assessments. There is also a facility for N et al. Guidelines for the diagnosis and management of indi- audio and video material. The success of any distance learning viduals with neurofibromatosis 1 (NF1). J Med Genet 2007; 44: programme is well-recognized to depend not only on the 81–8). We undertook a web-based survey among the mem- quality and clarity of the learning materials but also on sup- bers of the British Association of Dermatologists (BAD) to port for the students. Aside from administrative and technical assess management of NF1 and awareness of the NfA guide- support, online distance learners need different methods of lines. The 134 respondents (response rate 20%) included 72% support in learning. We provide learning support by individ- consultants, 19% specialist registrars and 8% from other ual synchronized tutorials online. Clinical cases are sent to the grades (such as associate specialists and clinical fellows). The students and then discussed, using the WimbaVoice tool in number of patients with NF1 reviewed within the last 12 WebCT that allows for ‘talk’ over the Internet. There is fur- months was low: none for 41% of respondents, 1–5 for 52% ther regular contact with the tutor by e-mail or telephone. In and 6 or more for 8%. Blood pressure would be regularly addition, a discussion board allows for asynchronous discus- recorded by 40% of respondents and a spine evaluation under- sion between students and tutors. To evaluate how students taken by 12%. A total of 62% would perform a skin examin- on the international programme perceive the level of support ation at each visit. Most did not refer to a NF1 coordinator

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 38 Bristol Cup Posters because there was not one locally (51%) or they were not and limbs. Liver and skin biopsies performed at this stage sure if one existed (27%). Twenty-two per cent of respond- revealed lobular chronic hepatitis and nonspecific inflamma- ents had access to a NF1 coordinator. Fifty-two per cent tion, respectively. Over the subsequent 4 months the infant would review patients with NF1 annually, 15% would dis- deteriorated and further cutaneous and liver biopsies were charge them following their initial visit, 21% were unsure performed. Histology from a truncal skin nodule revealed how frequently they would review them and 12% had other subcutaneous necrotizing granulomas, and granulomatous responses. Twenty-eight per cent considered that dermatolo- inflammation was confirmed in the liver; all special stains for gists should be the lead specialists for NF1 patients over 16 acid-fast bacilli and fungi were negative. There are 300 cases years old, followed by clinical geneticists (22%), general prac- of congenital tuberculosis in the world literature. Cantwell titioners (15%) and others (13%), but 22% were unsure. The et al. (Cantwell MF, Shehab ZM, Costello AM et al. Brief report: majority of respondents (76%) were not aware of the clinical congenital tuberculosis. N Engl J Med 1994; 331: 1051–4) guidelines published by the NfA. Of the 32 who were aware examined 29 cases and of these three had cutaneous lesions of them, 27 (84%) would comply with them. This survey attributable to the disease. The range of cutaneous lesions showed that there is limited knowledge of the NfA guidelines reported in congenital tuberculosis includes small flesh- among BAD members and, not surprisingly, their management coloured subcutaneous papules and nodules, cutaneous of patients with NF1 does not fully comply with them. This is plaques and necrotic papules. The variable appearance of the important as some view dermatologists as the specialists who cutaneous manifestations of congenital tuberculosis means the should have overall care of these patients. Greater awareness dermatologist’s index of suspicion must be high in order to of the guidelines would help to improve and standardize the make the diagnosis. In addition, dermatologists must have a management of NF1. Our survey has not only highlighted the low threshold for performing a skin biopsy and should send problem but also to some extent addressed it. samples for tuberculosis culture and microscopy in addition to histological analysis.

P-35 Cutaneous manifestations of congenital tuberculo- P-36 sis: case report and review of the literature Methicillin-resistant Staphylococcus aureus (MRSA) S. Bunting, R. Morris-Jones, S. Bansal and L.C. Fuller complicating skin disease mainly affects elderly Kings College NHS Trust, London, U.K. patients with eczema and leg ulcers who have The World Health Organization reports an increasing global associated chronic medical disorders burden of tuberculosis with 8Æ3 million new cases in 2000. A. Jayasekera, L. Jennings, C.R. Holden, C. Bates and Congenital tuberculosis is rare and poorly understood; how- D.J. Gawkrodger ever, its incidence is predicted to rise over the next decade Departments of Dermatology and Microbiology, Royal Hallamshire Hospital, with the advancing numbers of tuberculosis and HIV co-infect- Sheffield, U.K. ed individuals. Cutaneous manifestations of congenital tuber- Methicillin-resistant Staphylococcus aureus (MRSA) has had a huge culosis may be one of the earliest signs of infection. impact on healthcare. MRSA tends to affect elderly patients Dermatologists may therefore play an important role in aiding with chronic disease. In the dermatological setting, the litera- the early diagnosis of this often fatal disease by considering ture to date suggests patients with eczema and leg ulcers are the diagnosis when assessing newborns with failure to thrive, mostly affected. The aim of this study was to examine the hepatosplenomegaly and cutaneous lesions. A 2-day-old boy demographics of patients with skin disease in a hospital set- born at term to a South African woman presented to the pae- ting who became infected with MRSA, to look at the associ- diatricians with a fever. Physical examination revealed hepato- ated risk factors and to evaluate the effectiveness of treatment. splenomegaly and widespread lymphadenopathy. Liver Twenty-one patients with skin diseases who became infected function tests were abnormal and acid-fast bacilli were dem- with MRSA between January 2004 and December 2005 were onstrated on a gastric aspiration. Ultrasound scan revealed evaluated. In this period 58 585 patients were seen in the echo-dense areas in the liver and spleen consistent with milia- department. There were 13 women and eight men, 17 being ry tuberculosis. A diagnosis of congenital tuberculosis was older than 60 years. Sixteen were white British ethnic group. made; he was commenced on rifampicin, isoniazid and pyra- The skin diseases were eczema (7), leg ulcer (7), erythroder- zinamide. The infant’s mother was subsequently investigated ma (2) and bullous disease (1). In three subjects, MRSA and diagnosed with miliary tuberculosis and was also treated. caused a wound infection following surgery. One patient had HIV testing was negative for both mother and infant. Sputum primary MRSA infection () but in all the others the and gastric aspirate from mother and child, respectively, grew infection was secondary. All but one of the patients had MRSA fully sensitive Mycobacterium tuberculosis. At 3 months the infant phage type 15 and all isolates were of hospital-acquired types. presented with continuing failure to thrive and worsening Sixteen patients had a chronic disease, which was diabetes in jaundice. Examination revealed massive hepatosplenomegaly, six cases, with others having chronic obstructive airways dis- widespread lymphadenopathy, and multiple flesh-coloured ease and chronic renal failure. Seventeen patients had had hos- firm mobile subcutaneous papules and nodules on the trunk pital contact within a year of contracting MRSA and a third

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 39 were receiving long-term care. Anti-MRSA measures were presentation of tuberculosis occurring more than a year after taken in all inpatients. In six of eight inpatients, follow-up commencing infliximab, the extrapulmonary presentation and swabs were taken and in five cases, three clear swabs were also the good response to infliximab. Tuberculosis is a known obtained. For outpatients, anti-MRSA measures were taken in risk when using immunosuppression and has been reported in nine patients but it was unclear if measures were taken in the association with antitumour necrosis factor agents in particu- other four. In five, follow-up swabs were taken, but in no lar. Patients should be assessed for any prior history of tuber- case were three negative swabs obtained. This study shows culosis, undergo an appropriate clinical examination including that identified MRSA is relatively uncommon in patients chest X-ray and, if appropriate, a tuberculin test. The onset of attending a dermatology clinic. Patients who are affected tend tuberculosis is usually seen early in treatment within the first to be elderly with comorbidities, and their skin diseases are three infusions, unlike this case where presentation was after mostly eczema or leg ulceration. MRSA may cause wound in- eight infusions. More than half can have extrapulmonary dis- fection after surgery. Measures to treat inpatients were in the ease and a quarter have disseminated disease. Most are thought main satisfactory but anti-MRSA treatment of outpatients was to be due to reactivation of latent tuberculosis infection deficient, possibly due to a lack of resources or of good com- though some primary infections can occur. This case high- munication with community nurses. Further investigation into lights the need for continuous vigilance on the part of the cli- this is indicated. nician for the possibility of tuberculosis in patients treated with these agents, particularly in those patients in whom the tuberculin skin testing and the more recently available inter- P-37 feron-c based assays in detecting latent tuberculosis, are not Abstract withdrawn. applicable (such as immunocompromised patients). Under- standing optimal approaches to screening, monitoring and treatment is essential and stratification of these patients plays a P-38 key role in their screening and management. Pulmonary tuberculosis and choroidal granuloma in a patient with severe chronic plaque psoriasis treated with infliximab B.P. Amirtha Vani, P. Frith,* S.M. Downes,* P-39 S. Burge and H.M. Lewis Treatment of recalcitrant warts with topical University Hospital Birmingham NHS Trust, Sellyoak Hospital, Sellyoak, diphencyprone Birmingham, U.K.; *Oxford Radcliffe Hospitals NHS Trust, Oxford Eye A. Martin-Clavijo, K. Hotchkiss, C. Vlachou and Hospital, Oxford, U.K. and Oxford Radcliffe Hospitals NHS Trust, J. Berth-Jones Churchill Hospital, Oxford, U.K. University Hospital of Coventry and Warwickshire, Coventry, U.K. Tuberculosis and other opportunistic infections are a growing Recalcitrant warts resistant to common treatments such as concern with the expanding use of biological agents, in par- over-the-counter preparations or cryotherapy are frequent in ticular tumour necrosis factor inhibitors. We present the case dermatology departments and pose a challenging therapeutic of a 31-year-old white man affected with chronic plaque psor- problem. Topical immunotherapy with dinitrochlorobenzene iasis since the age of 8. He had undergone various conven- or diphencyprone (DPCN) has been used in the past with a tional treatments including psoralen plus ultraviolet A varying degree of success. We present here a review of our (PUVA), narrow-band UVB, ciclosporin, acitretin and metho- experience from 2000 to 2006. All patients were sensitized trexate. These treatments were stopped due to lack of response with 2% DPCN applied to the upper outer arm. Treatment or side-effects from therapy. Infliximab was commenced at a was carried out at 2-weekly intervals (increased to 3-weekly if dose of 5 mg kg–1 of body weight with concomitant metho- warranted by side-effects) and the concentrations adjusted se- trexate. Pretreatment investigations including a chest X-ray quentially according to response and side-effects (ranges from were normal. Tuberculin testing was not done as he was on 0Æ025% to 6%). Patients continued the treatment regime until immunosuppressive treatment. He had a good response to inf- complete resolution of the warts was obtained or until both liximab and his psoriasis was virtually clear by the fifth infu- patient and investigator were convinced there was no sion. More than a year after commencing infliximab and after response. Forty-eight patients were included in the study; eight infusions, he reported sudden onset of central visual dis- 67% of the patients were female and the mean age was 35 turbance in his right eye and he also reported a persistent years (range 9–72). Seventy-five per cent had hand warts cough, weight loss and malaise. Ophthalmological examin- (mean 8, range 1–30) and 62% feet warts (mean 4, range 1– ation revealed a choroidal granuloma with associated oedema. 21). The average duration of the warts was 4Æ1 years (range 6 Investigations including a chest X-ray and bronchoscopy and months to 15 years). Seventy-one per cent of the patients had lavage confirmed Mycobacterium tuberculosis complex infection. He received at least two other previous treatments; 25% of the was commenced on anti-tuberculous treatment and was trea- patients did not finish the treatment and were lost to follow- ted with prednisolone for the choroidal tuberculous granulo- up. These patients have been included in the intention-to-treat ma. We would like to highlight the following points: the late analysis. Twenty-five patients had complete responses (52%,

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 40 Bristol Cup Posters

68% of protocol correct) with only three patients having P-41 partial responses and eight patients failing to respond. From Value of p53 detection in common and genital warts the intention-to-treat group, 23% had six or fewer treat- A. Zidan, A.-R. Zekri, A. El-Ramly and K. El-Hoshy ments and 23% between seven and 12 treatments, with the Cairo University Teaching Hospitals, Cairo, Egypt rest needing more than 12 treatments. Looking at the com- p53 (the gate keeper or the executioner) is present in two plete responses, 12% cleared within six treatments (3 forms: the wild form which is a tumour suppressor gene and months), 52% within 12 treatments (6 months), 72% within cannot be detected immunohistochemically, and the mutant 18 treatments (9 months) and 88% within 24 treatments form which is an oncogene and can be detected immunohis- (12 months). Sixty-three per cent of the patients in this ser- tochemically. Human papilloma viruses (HPVs) comprise a ies had some side-effects, of which, itching (50%) and blis- large family of small double-stranded DNA viruses that can tering (33%) were the commonest. In conclusion, topical infect skin and mucous membranes causing mainly benign DPCN appears to be an effective treatment for resistant viral lesions. The aim of our work was to detect the level of p53 warts. It has considerable but tolerable side-effects and is, on expression in common and genital warts in order to identify the whole, safe. However, it may take up to 12 months for the role of apoptosis in regression, overgrowth or carcinogen- the warts to clear. esis of these lesions. A prospective study was done during the period from May 2002 to May 2004 on 30 patients (19 men, 11 women, median age 29 years). They were divided into two groups: group A included 20 patients with common P-40 warts and group B included 10 patients with genital warts. Treatment of recalcitrant viral warts with acitretin Patients were their own control. The duration of warts ranged A. Martin-Clavijo, I. Ahmed, F. Humphreys* and from 1 week to 30 years. Two skin biopsies were obtained J. Berth-Jones from each patient. An excisional biopsy of the warts was University Hospital of Coventry and Warwickshire, Coventry, U.K. and taken, which was then cut into two halves, the first half was *Warwick Hospital, Warwick, U.K. fixed in formalin, then paraffin embedded before being Viral warts are a common problem in the community. Some stained with haematoxylin and eosin to confirm the diagnosis. patients with extensive or resistant warts are referred to sec- This was followed by immunohistochemical studies on the ondary care for management. In cases where conventional, paraffin section using anti-p53 monoclonal antibodies. The over-the-counter preparations and cryotherapy have failed, other half of the excisional biopsy was stored at –80 C for treatments such as immunotherapy with topical diphency- DNA extraction and HPV typing. A second biopsy from the prone or intralesional bleomycin are used in our department. normal skin was taken and stored at –80 C for DNA extrac- We present a series of seven patients with viral warts who tion and HPV typing to find out whether the normal skin har- failed to respond to first- and second-line treatments, and bours the same HPV type or not. Results in group A showed were then treated with oral acitretin. The mean age was 52 that four of 20 lesions were positive for HPV types 11, 31, 6 years (range 20–75). Three were women and three were im- and 33, while one out of 20 normal skin biopsies was positive munocompromised (one post-renal transplant, one bone mar- for HPV type 33. Four out of 20 lesions showed positive p53 row transplant and one a CD4 lymphopenia of unknown staining. However, no cases were positive for both HPV and Æ origin). The mean duration of the warts was 11 6 years (range p53 staining. In group B, six of 10 cases were positive for 1–30). All but one had multiple warts. All had previously HPV types 6 and 11, while one out of 10 normal skin biop- been treated with over-the-counter treatments and cryothera- sies was positive for HPV type 5. Three out of 10 lesions py, three with diphencyprone, two with imiquimod, one with showed positive p53 staining, two of them were positive for intralesional bleomycin and one with cimetidine. The patients HPV. These results suggest that there may not be a direct rela- were started on oral acitretin after obtaining verbal consent tion between p53 positivity and HPV in common and genital and all were monitored appropriately. The mean dose was warts. However, larger studies are needed to elucidate the 30 mg daily (range 10–50 mg daily). The mean duration of mechanism by which HPV stimulates cell proliferation. the treatment is currently 9Æ7 months (range 1–30) and two of the patients are still undergoing treatment. In one case the treatment was discontinued after 1 month due to myalgia. The rest had no significant side-effects. Of the six remaining P-42 patients, three had a complete response and remain free of Treatment of recalcitrant warts with intralesional warts up to 3 years later. Three patients had a partial response, bleomycin two of these are still undergoing treatment, and one had treat- A. Martin-Clavijo, K. Hotchkiss, C. Vlachou and ment discontinued on diagnosis of Hodgkin’s lymphoma. In J. Berth-Jones conclusion, although retinoids are not commonly used in the University Hospital of Coventry and Warwickshire, Coventry, U.K. management of viral warts, they can play a useful role in the Recalcitrant warts resistant to cryotherapy and over-the-coun- treatment of selected cases, particularly in immunocomprom- ter treatments are a common event in dermatology depart- ised patients. ments and pose a challenging therapeutic problem. Multiple

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 41 treatments have been tried, including topical immunotherapy population of approximately 500 000 with 35% belonging to agents such as dinitrochlorobenzene or diphencyprone. Intra- ethnic minority groups. The ethnicity of all inpatients from lesional bleomycin has also been used with a varying degree April to December 2006 was 44% white (21 462), 4% Afro- of success. We present here a review of our experience of Caribbean (1733), 27% Asian (13 172) and 15% other or intralesional bleomycin from 2000 to 2006. Treatment was unknown. Sixty-five consecutive cases of acute lower limb cel- carried out at 2-weekly intervals (increased to 3-weekly if lulitis and 130 controls were recruited. Controls were matched warranted by side-effects) using 100 units of bleomycin mL–1 for age, gender and admission date (within 7 days). Research- of normal saline. A drop of this solution was placed over the ers were blinded to the patients’ ethnicity when selecting con- warts and then introduced in the lesions using a 25-gauge trols. There were 33 women (51%) in the cellulitis group and needle and multiple pricks. Patients continued this treatment the average age of this group was 71 years (range 24–93). regimen until the complete resolution of the warts or until There were 66 women (51%) in the control group and the both patient and investigator were convinced there was no average age of this group was 71 years (range 19–97). The response, but never before 4 months of treatment. Thirty-five ethnicity of the cellulitis group was 91% white (59), 6% patients were included; 62% were female and the mean age Asian (four) and 3% Afro-Caribbean (two). The ethnicity of was 42 years (range 15–73). Fifty-one per cent had hand the control group was 63% white (82), 23% Asian (30) and warts (mean 9 warts, range 1–50) and 51% feet warts (mean 14% Afro-Caribbean (18). Being white was a statistically sig- 6, range 1–15). The average duration of the lesions was 4Æ3 nificant risk factor for acute lower limb cellulitis (P <0Æ001). years (range 6 months to 18 years). Eighty-seven per cent of The results support the hypothesis that white ethnicity is a risk the patients had received at least two other previous treat- factor for lower limb cellulitis requiring admission to hospital. ments; 25% of the patients did not finish the treatment and Potential hypotheses explaining this imbalance include a were lost to follow-up. These patients have been included in genetic predisposition to cellulitis in the white group or a the analysis on an intention-to-treat basis. Twenty-two patients genetic resistance to cellulitis in the nonwhite groups. Alterna- had complete responses (69%, 91% of the protocol-correct tively, cultural issues that result in late presentation may occur patients) with only five patients failing to respond. From the in the white group or there may be differences in general intention-to-treat group, 16% had six or fewer treatments, practitioner referral practices. Differences may also exist 34% between seven and 12 treatments, 34% between 13 and between other ethnic groups although our numbers are too 18 treatments and 25% more than 24 treatments. Looking at small to investigate this further. Previous studies have identi- the complete responses, 14% cleared within six treatments (3 fied treatable risk factors for lower limb cellulitis, such as der- months), 41% within 12 treatments (6 months), 55% within matomycosis of the foot. By targeting high-risk groups such 18 treatments (9 months), 68% within 24 treatments (12 as white people, inpatient admissions of cellulitis may be pre- months) and 95% within 30 treatments (15 months). Only vented. Further investigation of this apparent disparity is war- 19% of the patients had side-effects such as pain (three ranted. This study was unfunded. patients) and itching (two patients). In conclusion, intrale- sional bleomycin appears to be an effective and safe treatment, well tolerated by patients. Although slow in action, it has a P-44 good success rate and should be considered a therapeutic A myriad of mycobacteria option for long-standing warts nonresponsive to other treat- C. Macedo, F.M. Pope, S. Karim, S. Barnass, ment modalities. A. Winning and S.C. Parker West Middlesex University Hospital, Isleworth, Middlesex, U.K. We describe six cases of cutaneous mycobacterial infection, P-43 which have presented to our department in the last 18 Ethnicity as a risk factor for acute lower limb cellu- months. Case 1: Mycobacterium marinum. A 35-year-old man pre- litis: a U.K.-based case–control study sented 5 years ago with a lump in his left breast. He subse- J. Halpern and N. Langford quently had 10 admissions with recurrent abscesses and City Hospital, Birmingham, U.K. cellulitis, and one episode of tendonitis, before he was Acute cellulitis of the lower limb is a common disease in the referred to dermatology when he had acute cellulitis of his U.K. It often results in hospitalization and long-term morbid- right leg, a cold , and scattered granulomatous and ity. Previous case–control studies suggest that lower limb sporotrichoid lesions. He kept tropical fish. M. marinum was oedema, tinea pedis and ulceration are potential risk factors confirmed on culture and polymerase chain reaction analysis. for cellulitis. Being of white ethnicity may also be a risk fac- Case 2: M. marinum. A 23-year-old woman presented with a 3- tor, although this has not been directly assessed. We under- month history of sporotrichoid lesions on her right forearm. took a prospective case–control study to determine whether She also kept tropical fish. Histology showed granulomata and white ethnicity is a risk factor for acute lower limb cellulitis she responded to doxycycline. Case 3: M. leprae. A 35-year-old in the U.K. Local Research Ethics Committee approval was Brazilian woman presented with painful erythematous nonspe- obtained prior to the study. The study was undertaken in the cific lesions on her elbows and feet 6 months after the birth of admissions unit of a 700-bed inner city hospital serving a her first child. Initial biopsy was nonspecific. She subsequently

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 42 Bristol Cup Posters developed scaly anaesthetic plaques on her limbs and a painful Carotene Trial (50 mg daily). The pooled odds ratio (OR) for enlarged ulnar nerve. Repeat biopsy showed granulomatous these studies was 1Æ00 (0Æ94, 1Æ07) for basal cell carcinoma inflammation with neurotropism. She was diagnosed with (BCC) and 1Æ07 (0Æ89, 1Æ30) for squamous cell carcinoma upgrading borderline tuberculoid . Case 4: M. fortuitum. (SCC). Selenium supplementation was reported in one RCT A 27-year-old housewife from India presented with a large (200 lg daily); the risk in the intervention group for BCC OR abdominal mass with discharging sinuses. It had appeared 1Æ1(0Æ95, 1Æ28) and SCC OR 1Æ14 (0Æ93, 1Æ39) and melanoma shortly after a Caesarean section in India, over 1 year previ- OR 1Æ18 (0Æ49, 2Æ85). Two RCTs reported the risk of retinol ously. She had a needle aspiration while in India, and was supplements (25 000 IU daily) for BCC OR 1Æ10 (0Æ90, 1Æ34) told she had a desmoid tumour. Our biopsy showed granulo- and SCC OR 0Æ72 (0Æ55, 0Æ95). Two cohort studies show some matous inflammation with necrosis, highly suggestive of evidence of increased risk of melanoma with increased body M. tuberculosis infection. Initial culture was negative. She failed surface area OR 1Æ10 (1Æ08, 1Æ03) per 0Æ1m2 and standardized to respond to standard antituberculous therapy and repeat mortality ratio 1Æ9(1Æ0, 3Æ7) for > 1Æ96 vs. £ 1Æ68 m2. There culture grew M. fortuitum. Case 5: M. tuberculosis. A 23-year-old was little evidence of a similar association for weight, obesity or man from India, presented to the orthopaedic surgeons with a body mass index. Fourteen RCTs of dietary interventions have 2-month history of a large discharging ulcer on the anterior reported on skin cancer – more than for any other anatomical aspect of his right ankle. Biopsy showed caseating granulomata site. Evidence is lacking to support the hypothesis that dietary and M. tuberculosis was grown. Case 6: M. tuberculosis. A 52-year- modification prevents skin cancer. The large personal expendi- old woman from Somalia presented with multiple fluctuant ture on supplements demonstrates the need to understand their subcutaneous pus-filled abscesses. She also gave a history of effects on cancer prevention so that people can be better weight loss, fevers and night sweats. A computed tomography informed. Aspects of this data have been presented in Nice. scan showed deposits in her spleen and intraperitoneally. Funding: World Cancer Research Fund. Review team members: Histology of one of the abscesses showed granulomata, and R. Beynon, T. Bekkering, M. Burke, G. Davey Smith, R. Harbord, culture confirmed M. tuberculosis. In summary, it is important to R. Harris, A. Ness, M. Pignatelli, J. Savovic, J. Sterne, S. Thomas, be aware of the wide-ranging nature and varying presentations L. Zuccolo. External advisors: C. Bain, D.A.R. de Berker, A. of the different mycobacterial infections. Diagnosis has some- Green, L. Hooper. times been difficult either due to nonclassical presentation, or problems in culturing the organism or because initial presen- tation has been to nondermatologists. P-46 A study of sun avoidance and protection measures in primary schools in the south east of Ireland P-45 M. Laing, J. Sui and C.C. Buckley A systematic literature review of the effects of diet Waterford Regional Hospital, Waterford, Ireland on skin cancer Childhood exposure to ultraviolet (UV) radiation from the sun S. Thomas, J. Sterne,* R. Beynon* and and a history of sunburn are risk factors for skin cancer. School D. de Berker policy addressing sun protection is an important issue. Residents Division of Maxillofacial Surgery and *Department of Social Medicine, Bristol in south east Ireland are at high risk for UV radiation exposure. University, Bristol, U.K. and Bristol Dermatology Centre, Bristol Royal To understand better sun-protection policies and practices in Infirmary, Bristol, U.K. schools in the south east of Ireland a sample of 345 primary A systematic review of the role of diet in skin cancer risk was schools were surveyed. The aim of our survey was to determine undertaken as part of a larger review of both diet and physical whether a sun-protection policy existed in these schools. We activity on all cancers, funded by the World Cancer Research also aimed to determine the current school practices in sun pro- Fund. We searched for literature by standard systematic review tection and teachers’ opinions about the importance of sun pro- methods searching online databases: Cochrane library, MED- tection in schools. A list of schools was obtained from the LINE, EMBASE and AMED. We used medical subject headings Department of Education website. School sizes varied from nine (MeSH terms) and reference lists of relevant books, reviews and to 425 pupils. The questionnaire was posted to principals of publications located. Random-effects meta-analysis was conduc- schools. Of 345 distributed questionnaires, 274 were returned. ted by the method of der Simonian and Laird, and quantified Ninety-five per cent of schools had no written policy to limit heterogeneity using the I2 statistic. A total of 21 272 studies students’ sunlight exposure. One hundred per cent of schools were found; of these 751 were potentially relevant and 148 met had outdoor activities scheduled between 10Æ00 and 14Æ00. the inclusion criteria. It was not possible to estimate a dose Forty-four per cent of schools had no shade-producing struc- response in 50% of results. The large majority of studies were tures. The school uniform comprised of hats in 24%, short pants conducted in the U.S.A. and vitamins were the exposure most in 38% and short sleeves in 66% of schools. Twenty-two per commonly reported. The role of antioxidants was investigated. cent of school personnel (including teachers, PE teachers, school Three randomized controlled trials (RCTs) reported on beta- nurses) never wear hats, long sleeves or sunglasses, or use carotene supplementation: the Physicians Health Study (50 mg umbrellas. Sunscreen was encouraged in only 55% of schools, alternate days), the Nambour Study (30 mg daily) and the Beta- mainly on school excursions. Although no sun-protection policy

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 43 exists in schools, 80% of principals and teachers feel this is out of the 31 replies, a significant proportion have seen a required. One per cent of teachers felt that sun exposure during melanoma develop at the site of what was clinically or histo- childhood is not an important health concern. In summary, logically diagnosed as a Spitz naevus. Clearly it is important 100% of schools had outdoor activities scheduled between that further studies are performed to determine the optimal 10Æ00 and 14Æ00. Forty-two per cent of schools had no shade- management of clinically obvious Spitz naevi in children. producing structures. Ninety-five per cent of schools had no documented sun-protection policy although 80% of principals and teachers feel this is required

P-48 Breaking bad news to patients with melanoma P-47 L. Williams and J. Garioch Survey on the management of Spitz naevi in children Norfolk and Norwich University Hospital, Norwich, U.K. by Irish dermatologists Trust guidelines have been developed concerning how bad C. Feighery, M. McCarron and E.A. Bingham news should be broken to patients. These were developed fol- Royal Hospitals Trust, Belfast, U.K. lowing the King’s Fund study on breaking bad news (Walker There is no consensus regarding the management of Spitz G, Bradburn J, Maher J. Breaking Bad News: establishing an auditable naevi. In children they are regarded by many as benign. A procedure for giving the cancer diagnosis. London: The King’s Fund, 2002 survey of dermatologists in the U.S.A. found that 50% 1997). The guidelines cover five aspects of the consultation: of paediatric dermatologists would treat Spitz naevi differently the environment, the people involved, the verbal information in children compared with adults (Gelbard SN, Tripp JM, given, the amount of information given, and the information Marghoob AA et al. Management of Spitz naevi: a survey of and support offered. The aim of this audit was to determine if dermatologists in the United States. J Am Acad Dermatol 2002; these guidelines are met when the news of a diagnosis of ma- 47: 224–300). Most US dermatologists tend to excise these lignant melanoma is broken to patients. A standard of 90% lesions in children. Our aim was to survey the dermatologists was set. All the patients whose biopsy proved malignant mela- in Ireland to ascertain how Spitz naevi in children (age < 12) noma in September 2005 were identified from the patient are managed here. The practising dermatologists in Ireland administration system. These patients were sent a question- were identified from the Irish Association of Dermatologists naire asking them about the experience of receiving their mailing list. A one-page anonymous questionnaire was posted. diagnosis. Thirty-seven questionnaires were sent out and 27 Questions included whether or not dermatologists would replies were received. The questionnaires were anonymous. In biopsy (excision/punch) what was clinically a Spitz naevus, general, patients were happy with the environment: the stand- whether they would consider a pigmented Spitz naevus more ard was met in five out of the six questions. They were satis- suspicious than a nonpigmented naevus and if they consider fied with the way the doctor behaved during the consultation. these lesions benign or precursors to melanoma. They were However, the standard was only met in three out of the eight also asked about the follow-up period and whether they had questions asking about the people involved, either because a experience of a melanoma arising at a site of a lesion initially nurse was not present or because they were insufficiently diagnosed as either clinically or histologically a Spitz naevus. involved. Patients were happy with the verbal information The total number of questionnaires sent was 48. Of these, 37 they received, with the standard being met in four out of six were returned giving a reply rate of 77%. Six of these were questions. The standard was only met in one out of five ques- returned unanswered from dermatologists who do not see tions asking about the amount of information given because children. Results showed that 48% (15) of those surveyed opportunities were not offered for further discussion after the would biopsy what is clinically a Spitz naevus. Of these 87% immediate consultation. The biggest failing was found in the (13) would perform an excision and 46% (7) would not con- category of information and support where the standard was tinue to follow-up these children. Forty-nine per cent (15) felt not reached on any of the nine questions. This was because of that pigmented Spitz naevi were more suspicious than non- the paucity of written information available. There are many pigmented naevi. Of the dermatologists who do not excise aspects of difficult consultations that are done to patients’ sat- clinically obvious Spitz naevi in children, 67% (12) would isfaction. The biggest area of failing standards, as illustrated by follow-up for 3 years or more. Overall 87% (27) of those sur- this audit, is the distribution of written information. As a veyed felt that Spitz naevi in children are benign. Nobody felt result of our findings, we have developed a patient Filofax that it is a precursor to melanoma in children. Ten per cent note (Filofax UK, Burgess Hill, U.K.) containing relevant in- (three) have seen a melanoma in childhood arise at a site of a formation: names of those present during the consultation; lesion initially diagnosed clinically as a Spitz naevus and 13% various contact details; information on malignant melanoma (four) have seen a melanoma arise at a site of a lesion histo- and personalized management plans. We plan to distribute the logically diagnosed as a Spitz naevus. This survey has shown Filofax to those with newly diagnosed melanoma. We aim to that the treatment of what is clinically a Spitz naevus in chil- re-audit in a year’s time to see if the Filofax fulfils patients’ dren varies considerably between dermatologists. Worryingly, information needs.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 44 Bristol Cup Posters

P-49 P-50 Diagnosing melanomas: are things improving? A case of multiple, localized, eruptive keratoacan- R. Meys, G. Smith and V.R. Doherty thomas appearing at a site of resolved cellulitis Department of Dermatology, Royal Infirmary, Edinburgh, U.K. C. Groves, C. Fahy, M. McMenamin and L. Barnes Diagnosis of a melanoma while Breslow thickness is ‘thin’ is St James’ Hospital, Dublin, Ireland associated with good prognosis (Breslow A. Thickness, cross- Keratoacanthomas are tumours arising from the sebaceous sectional areas and depth of invasion in the prognosis of cuta- glands that will regress in the vast majority of cases. They neous melanoma. Ann Surg 1970; 172: 902–8). Approaches manifest as rapidly growing nodules with a characteristic cen- such as public or professional education and designated rapid- tral, keratin-filled crater. They can invade locally causing tissue access clinics have been utilized to achieve this goal and also damage and significant scarring. They are usually solitary; to meet government cancer target waiting times. We review accounts of spontaneous eruptive multiple lesions are a rarity. the recent performance of our departmental melanoma diag- Here, we discuss one such case. We report a case of multiple nosis and treatment service. All histologically diagnosed mela- keratoacanthomas appearing at the same time on the right nomas in our centre in 2005 were identified from our lower leg of a 79-year-old woman. The patient had been regional database. Patient demographics, pathology details, admitted to our hospital 9 weeks prior to the tumour appear- date of treatment(s) and referral pathway details were recor- ance for management of a cellulitis affecting the right lower ded. The type of clinic making the diagnosis was also noted leg. The cellulitis was bullous in nature and difficult to treat. and subdivided into a melanoma assessment clinic (MAC), Treatment with four antibiotics was required to manage the other tumour clinics (TC), general dermatology clinics (DC) streptococcal infection successfully. She was reviewed by the and general practitioner (GP) diagnosis. The MAC works as dermatology team while an inpatient and lesions suspicious of rapid access by triage of referral or by use of a specific mela- keratoacanthomas were not noted. The six lesions grew rap- noma proforma. The TC selects by letter triage of likely non- idly into nodules measuring between 0Æ5 and 3 cm in diam- melanoma tumours and the DC would take the remainder of eter before showing signs of spontaneous involution after 8 lesion referrals. One hundred and twenty-five melanomas weeks. There was no history of excessive ultraviolet (UV) were diagnosed and/or widely excised in dermatology in radiation or carcinogenic chemical exposure. The skin biopsy 2005. Twenty-nine (23%) were in situ only, 47 (38%) were of one lesion was in keeping with a diagnosis of keratoacanth- invasive but thin (< 1 mm Breslow thickness) and 42 (34%) oma. Serology confirmed full immunocompetence with an were thick, i.e. ‡ 1 mm. Sixteen cases (13%) were initially absence of features suggestive of an underlying haematological excised in primary care and 11 (9%) were diagnosed as inci- malignancy. Multiple keratoacanthomas are rarely seen; they dental findings in secondary care. Within the dermatology are a feature of Grzybowski’s generalized eruptive keratoacanth- department, 42% of melanomas were diagnosed at the MAC, oma, multiple eruptive keratoacanthoma of Witten and Zak, 22% at the TC and 19% at the DC. For invasive lesions only, the clinical entity described by Ferguson-Smith and also in as- range, mean and median Breslow in mm were, respectively, sociation with Muir–Torre syndrome. Our patient could not for MAC 0Æ3–3Æ2, 0Æ95 and 0Æ7, for TC 0Æ35–5Æ1, 1Æ54 and be categorized by any of the above syndromes. The precise 0Æ85, for DC 0Æ6–20, 2Æ09 and 1Æ5 and for GP 0Æ3–3Æ5, 1Æ19 aetiology of keratoacanthomas is unknown. Proposed factors and 1Æ2. Mean ages for melanoma patients at each clinic type include: viral infection (HPV), excessive UV exposure, immu- in same order were 55Æ9, 59Æ7, 60Æ5 and 43Æ6 years. Thus nodeficiency, chemical carcinogen exposure, scarring pro- the rapid-access MAC clinic seems to see thinner invasive and duced by chronic skin conditions, dysregulation of tumour more in situ lesions in a slightly younger patient group than suppressor or cellular oncogenes and skin trauma. Reports of other hospital clinics. This is likely to reflect the use of a pro- keratoacanthomas following skin trauma are rare but include forma-based referral which favours classic early superficial thermal burns, cryotherapy, laser resurfacing, radiation ther- spreading lesions rather than helps to select thicker or atypical apy, lacerations, post-skin grafting, post-skin cancer excision, lesions. In primary care there remains a lack of confidence in vaccination and arterial blood gas sampling. There have been melanoma recognition with diagnosis largely unsuspected no previous reports of multiple, eruptive keratoacanthomas prior to excision. The reason for the significantly younger occurring at a site of a resolved cellulitis. melanoma patient group in primary care is not clear. In 35% of hospital-referred cases no level of urgency was stated by P-51 the GP. In conclusion, we present data on melanoma diagno- Is it mandatory to follow up thin malignant sis for our region. While the MAC does see the largest num- melanomas? ber of melanomas in a shorter time it concerns us that A. James, U. Zaidi and R.M. Graham thicker lesions remain difficult to diagnose. Consideration James Paget University Hospital Foundation NHS Trust, Gorleston, Great needs to be given to improved recognition, both in primary Yarmouth, U.K. care and at hospital triage, of thicker melanomas which con- Malignant melanoma is the major cause of death from skin tinue to form a significant component of all dermatology cancers. Although the risk of recurrence and distant metastasis department workloads. of malignant melanomas can be predicted by a variety of independent risk factors such as sex, ulceration or site, the

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 45 single most commonly used predictor is the Breslow thickness. metastatic skin nodule. Further nodules appeared but interven- There is a direct relationship between Breslow thickness and tion was delayed until after delivery of a healthy boy at term prognosis of the disease. We conducted a retrospective study who is disease free at 21 months of age. During the next 12 to ascertain the need for follow-up of patients with malignant months several metastatic cutaneous and subcutaneous nodules melanoma with a Breslow thickness of 0Æ75 mm or less for 5 were excised from different body sites. The patient com- years who attended the outpatient clinic between 1999 and menced intramuscular progesterone injections for contracep- 2000. A total of 107 patients satisfied these criteria. In this tion. Gross examination of the placenta was normal in all three study, 62% (n = 66) were women and 38% (n = 41) were cases; none of the placentas were examined microscopically. men, with a median age of 54 years. Regarding the site of Pregnancy does not significantly increase the risk of developing lesions, 36% were found in lower limbs, 34% in the trunk, MM or affect survival rates; prognosis still depends on the fea- 17% in upper limbs and 13% in the head and neck area. His- tures of the primary tumour (Driscoll MS, Grant-Kels JM. Nevi tologically, 70% of the lesions were superficial spreading and melanoma in pregnancy. Dermatol Clin 2006; 24: 199–204). melanomas, 7% were lentigo maligna and the rest of the Data on disease-free interval are conflicting. Pregnant women lesions were desmoplastic, spindle cell, acral lentiginous, nod- generally had thicker MM at the time of diagnosis. Where pos- ular and invasive types. When the excision margins were com- sible, pregnancy should be delayed for 2–3 years following a pared 87% had complete excision and 10% had incomplete MM because of the risk of recurrences during this period. excision. There was no mention of the excision margins in There is no evidence of risk associated with hormonal contra- 3% of the cases. Less than 1% of subjects had local recurrence. ception and MM. Guidelines are needed to ensure that in preg- There were no deaths related to melanomas during the 5-year nant women with metastatic MM, examination is undertaken follow-up period. The development of new melanomas in this of the placenta (gross and histological) and of the child at group was 5%. Therefore, our study revealed that follow-up birth, for at least 2 years to exclude metastases. of thin melanomas for a 5-year period failed to show any sig- nificant recurrence and raises the question of whether these P-53 patients could be followed up in primary care? Do coal tar shampoos cause cancer? A systematic review P-52 S.W. Langan and H.C. Williams Malignant melanoma and pregnancy: what are the University of Nottingham, Nottingham, U.K. facts? Coal tar shampoos are a well-established component of both R. Katugampola and C. Mills prescription and over-the-counter (OTC) management of dis- Department of Dermatology, Royal Gwent Hospital, Newport, U.K. orders associated with scalp scale, in particular, psoriasis. The The incidence of malignant melanoma (MM) is increasing in role of coal tar as an occupational carcinogen has been estab- women of childbearing age. Of the 1 in 1000 pregnancies lished since the work of Percival Pott in 1775. There has been associated with malignancy, MM is the sixth most prevalent, concern over the possibility of carcinogenicity with the use of and the commonest metastasis is to the placenta and fetus. therapeutic tar for decades leading to banning of these prod- Three cases illustrate some issues surrounding MM in preg- ucts in some European countries. Is there enough evidence of nancy. Case 1: a 22-year-old woman at 16 weeks’ gestation carcinogenicity to ban the use of tar shampoos in the U.K.? A presented with a 6-week history of a changing mole on the systematic review was carried out using MEDLINE to look for face. Histology confirmed a MM (Breslow: 3Æ5 mm). A healthy relevant articles. We included experimental evidence and evi- girl born at full term remains disease free at 5 months of age, dence from various types of epidemiological studies, exclu- as is the patient. Case 2: the histology of a 36-year-old nullip- ding case reports and anecdotal reports. Our search identified arous woman with a few months history of a changing lesion 125 articles of which 16 were relevant. Data from rodent on the right calf confirmed a MM (Breslow: 3Æ6 mm). At 2 studies show that the presence of polycyclic aromatic hydro- weeks follow-up she was 4 weeks pregnant. At 24 weeks’ ges- carbons (PAH) in coal tar is associated with skin carcinogenic- tation a 2 cm right inguinal lymph node was noted but inter- ity, particularly when combined with either ultraviolet (UV) B vention was delayed until delivery. A healthy girl born at 38 radiation or dithranol. There is limited evidence that use of tar weeks’ gestation is disease free at 14 months of age. Six shampoos in human volunteers is associated with increased months postpartum the patient underwent excision of cerebral PAH levels. Other studies suggest that after a month of con- metastasis and a right inguinal block dissection (1 of 9 nodes tinuous shampoo use, the PAH levels reduce to baseline val- positive for MM). Fourteen months postpartum the patient ues. One of the major studies supporting the skin remains free from further recurrence. Case 3: a 33-year-old carcinogenicity of tar in patients with psoriasis did not separ- nulliparous woman had a MM (Breslow: 2 mm, Clarke’s ate the use of tar from the use of UVB as used in the Goecker- level 3) excised from the left scapular area, followed by left man regime. That study showed an increase in cutaneous axillary clearance 1 year later (1 of 26 nodes positive for MM). squamous cell carcinomas in patients treated with high doses Twenty-six months after excision of the primary MM she of psoralen plus UVA (PUVA) combined with either tar and/ became pregnant and remained well during the first and sec- or UVB, RR 4Æ5 (95% CI 1Æ3–16Æ1). Many other studies have ond trimester. At 30 weeks’ gestation she developed a distal failed to show this association, although most studies have

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 46 Bristol Cup Posters incompletely adjusted for confounders or are of too short a ness. Most patients in the study were retired and only two follow-up period to truly assess this association. Data from faced more time off work than anticipated and none felt their occupational studies suggest that there may be a lag period of scar would have limited their career advancement if they were 30–40 years before cancer develops. No study has directly working. Most patients felt their outdoor activities were not assessed the therapeutic use of tar shampoos using the devel- limited after Mohs’ surgery due to the defect. Some patients opment of cancer as an outcome measure (as opposed to PAH did comment that their friends and family would mention the levels which may be an unreliable proxy measure). Studies scar. Only two patients remained isolated for 1 week or more. need to be done which can assess the effects of tar separately Most patients reported positively about their experience and from the effects of UVB, PUVA and smoking. These studies outcome from Mohs’ surgery. In conclusion, assessment of need to be randomized, double-blind placebo-controlled stud- and seeking consent from patients for Mohs’ surgery needs to ies in patients with scalp psoriasis. Several outcome measures be structured and tailored to allow for psychosocial implica- including proxy measures need to be used to assess risk as the tions experienced by patients particularly with regard to facial primary outcome may be associated with a long lag period. If disfigurement and the appearance of the scar. these studies demonstrate even a small increase in the relative risk of cancer allowing for confounding factors, use of these products should be banned as small amounts of risk affecting P-55 large numbers of people is a significant public health issue. Is the incidence of malignant melanoma continuing to increase in the south west of the U.K.? A. Thomson, R. Corkill* and F. Bishr P-54 Royal Cornwall Hospital, Truro, Cornwall, U.K. and *Derriford Hospital, Patient quality of life after Mohs’ micrographic surgery Plymouth, Devon, U.K. S. Gossain and S. Keohane The incidence of malignant melanoma has risen in the U.K. Dermatology, St Mary’s Hospital, Portsmouth, U.K. three- to fourfold in the past 25 years, with the South West Eighty per cent of nonmelanoma skin cancer (NMSC) occurs region continuing to have among the highest levels in the on the head and neck and treatments can result in significant country. The Chief Medical Officer highlighted this problem in morbidity for patients because of the cosmetic and functional a 2003 report (www.dh.gov.uk). This analysis was performed importance of this area. The aim of this study was to investi- to establish updated figures for the incidence of malignant gate patients’ quality of life after Mohs’ micrographic surgery melanoma in Cornwall for 2005, and to investigate whether for NMSC on the head and neck with particular regard to the rising trend has continued in this region compared with the patients’ expectations of the size and depth of the scar and 2003 data. The pathology database of the Royal Cornwall Hos- postoperative morbidity due to this. Psychosocial and informa- pital was searched electronically using histological classification tion needs of the patients were also explored. We used a val- codes for all diagnoses of malignant melanoma from pathology idated disease-specific quality of life instrument, the Facial specimens received in 2005. The pathology reports were then Skin Cancer Index (FSCI). Semistructured interviews based on reviewed to ensure accuracy of the data and compared with the FSCI were conducted with 20 patients who had undergone previously recorded figures. In 2003 the incidence of malignant Mohs’ surgery in the previous 3–12 months. Six main melanoma in the U.K. was 13Æ4 per 100 000, and in the South domains were enquired about: emotional/anxiety; scar West was 22Æ7 per 100 000. We discovered that there were appearance/size and depth; embarrassment due to scar/ 134 cases of malignant melanoma in 2005 in Cornwall. Given decreased personal attractiveness; work/financial implications/ a population of 500 000 for the county, the incidence for ma- time off/lack of career advancement due to scar; lifestyle and lignant melanoma in 2005 is 26Æ8 per 100 000, a rise of 18% recreation (limits on outdoor activities due to scar site and compared to 2003. These new data provide evidence for per- size); and social/family attitudes/isolation period and physical sistently high levels of cases of malignant melanoma in Corn- functioning (soreness at site). Demographic data were also wall and appear to demonstrate a further significant rise in the collected for each patient including age, sex, marital status, incidence of such cases in the South West region of the U.K. employment status, type and location of skin cancer. Twenty Possible reasons for this include increased detection and treat- patients were interviewed; the commonest tumour type was a ment, improved recording of cases, changing population char- basal cell carcinoma (BCC) and the nose and cheek were the acteristics and a true rise in incidence. The figures may, commonest sites for Mohs’ surgery. The age range of the however, be an underestimate of the true problem. If mela- patients interviewed was 55–76 years. Most patients reported noma cases did not receive a biopsy or surgical intervention, mild to moderate anxiety prior to Mohs’ surgery and mild were treated out of the area, or if the incorrect histological code anxiety of facial disfigurement. Most patients reported mild to was used, then they would not be recorded or identified from moderate concerns with regard to their appearance 3–12 the database. It is clear, however, that the incidence does months after Mohs’ surgery. Patients expressed views such as remain high and appears to be increasing in the South West of consciousness about the scar, the scar being larger than antici- the U.K. It is therefore important that the efforts to improve pated by the patient, size of the defect and embarrassment, the management of malignant melanoma through research, and three patients perceived a reduction in personal attractive- prevention, diagnosis and treatment continue.

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P-56 presence of antecedent, synchronous or subsequent malignan- Sun-protection policies in primary and secondary cies in a cohort of melanoma patients recruited from our schools compared with ‘SunSmart’ guidelines melanoma database. A retrospective analysis was performed on S. Hoque, N.C. Chetty, W. Dudley and C.C. Harland a selection of patients with histologically confirmed malignant Epsom and St Helier University Hospitals NHS Trust, Carshalton, Surrey, melanoma from 1998 to 2006. Of all 667 melanoma patients U.K. in our database, we only had adequate information on other The incidence of skin cancer in the U.K. continues to rise. malignancies available in 200 patients and their first-degree Acute sunburn, particularly in childhood is one of the main relatives. A histologically validated national cross-reference an- risk factors for the subsequent development of skin cancer. alysis was carried out on the commonest noncutaneous Several studies in Australia have shown that school-based pro- tumour related to melanoma, which was breast carcinoma. grammes do improve sun-awareness knowledge and can There were 65 men and 136 women. Twenty-seven had meta- potentially modify behaviour. Cancer Research U.K. has issued static melanoma of which 16 died. Two per cent had a second ‘SunSmart’ guidelines aimed at nurseries, pre-schools and pri- primary melanoma. Six per cent had a family history of mela- mary and secondary schools to encourage the development of noma. Twenty-one squamous cell carcinoma and basal cell sun-protection policies. Based on these protection measures, a carcinoma were identified in 14 patients with melanoma. questionnaire was sent to 342 local nurseries and schools. The Twenty-two noncutaneous malignancies were identified in this response rate was 50% (73 of 147) for primary schools (PS), cohort of patients and their first-degree relatives (five men, fif- 53% (24 of 45) for secondary schools (SS) and 15% (23 of teen women). In order of frequency, the number of noncuta- 150) for nurseries; nurseries were omitted from further analy- neous malignancies detected were: four breast carcinoma, sis. Results showed that sun protection is actively promoted to three colorectal carcinoma, three squamous cell carcinoma of pupils via, for example, assemblies and workshops in 67% of the lung, two endometrial carcinoma and one case of each of PS and 58% of SS. Trees have been planted for long-term the following: lymphoma, renal cell carcinoma, transitional shade in 59% of PS and 63% of SS but plans to improve meas- cell carcinoma of the bladder, oat cell carcinoma of the lung, ures utilizing shade were infrequently in place (18% of PS; gastric carcinoma, oesophageal carcinoma, follicular thyroid 17% of SS). Outdoor play was scheduled outside of 11Æ00 to carcinoma, primary brain carcinoma, cervical carcinoma and 15Æ00 wherever possible in 21% of PS and 29% of SS. Pupils prostate carcinoma. Breast carcinoma having the highest asso- were encouraged to wear wide-brimmed or legionnaire-style ciation with melanoma led us to perform a cross-reference an- hats when outside in 85% of PS but only 38% of SS. A stock alysis from the National Irish Cancer Registry of all of school sunscreen for children who have forgotten or cannot melanomas and breast carcinoma patients in Ireland. Using afford their own is available in 22% of PS and 21% of SS. the current number of patients with breast carcinoma and Overall, the results indicate that ‘SunSmart’ guidelines are cur- applying the incidence rate for melanoma, or vice versa, there rently being implemented poorly and, worryingly, most of should only be 20–35 patients with both cancers if they were the schools which replied do not have plans in place to not related. Our national database revealed 127 cases, giving a improve on these key points. More publicity and awareness fourfold increased risk in the number of observed cases than needs to be targeted specifically at head teachers and leaders expected. Our local figures show a high prevalence of second of parents associations, particularly in SS, which fared worse malignancies in malignant melanoma. Nationally, breast carci- in the survey. Inevitably this requires greater allocation of noma increased fourfold. These data suggest that patients with resources which are already limited. Nonetheless, implementa- melanoma and breast carcinoma should be carefully screened tion of ‘SunSmart’ strategies among nurseries and schools for coincidental occurrence of these malignancies and possibly deserves greater prioritization. other systemic malignancies. Genetic factors may explain these associations. Surveillance for other cutaneous malignancies is also warranted. P-57 Malignant melanoma and its association with other cutaneous and noncutaneous malignancies P-58 W.L. Ho and G.M. Murphy Autosomal dominant chronic mucocutaneous Department of Dermatology, Beaumont Hospital, Dublin, Ireland and hypothyroidism complicated by Melanoma is a complex disease that has host-related, genetic oesophageal carcinoma and environmental factors contributing to its aetiology. Second D. Koch, D. Lilic* and A.J. Carmichael malignancies in melanoma patients have been reported with Department of Dermatology, The James Cook University Hospital, Middles- variable frequency (Schmid-Wendtner M-H, Baumert J, brough, U.K. and *Institute for Cellular Medicine, The University of Wendtner C-M et al. Risk of secondary primary malignancies Newcastle upon Tyne, Newcastle upon Tyne, U.K. in patients with cutaneous melanoma. Br J Dermatol 2001; 145: We report three generations of a family with autosomal dom- 981–5; Wu YH, Kim GH, Wagner JD et al. The association inant mucocutaneous candidiasis (CMCC) associated with pri- between malignant melanoma and noncutaneous malignancies. mary hypothyroidism and oesophageal carcinoma in the index Int J Dermatol 2006; 45: 529–34). The aim was to evaluate the case. Patient 1, a man, developed candidiasis at the age of 4

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 48 Bristol Cup Posters years affecting the oropharynx, nails and oesophagus. From skin disorders, including skin malignancies. All new referrals the age of 38 years he was treated with long-term oral keto- attending a specialist dermatology ‘lumps and bumps’ clinic conazole, which controlled his . At 32 years of over a 2-month period were included. The clinician seeing the age he was diagnosed with primary hypothyroidism. At the patient filled out a specially designed questionnaire. Variables age of 42 years he was diagnosed with a well-differentiated assessed were the presence or absence of trauma and its nat- squamous cell carcinoma of the oesophagus, from which he ure, the temporal relationship to the presenting problem and died 5 months later, having been a life-long nonsmoker. His whether the history of trauma was volunteered by the patient parents and nine siblings did not have CMCC or associated or elicited by the clinician. Clinicopathological correlation of endocrinopathies. He had three children, of whom his daugh- diagnosis was made in all malignant cases, and where avail- ter, patient 2, and younger son, patient 3, have CMCC. Patient able, in others. A total of 151 patients were seen. Of these, 2 developed persistent oral candidiasis at 6 months of age and 113 (75%) were included in the analysis. Patients with an subsequently vaginal and oesophageal involvement. From the inadequately filled questionnaire were excluded. The most age of 13 years she has had recurrent candidal infection of the common diagnosis was basal cell carcinoma (BCC) in 24 finger nails requiring repeated nail avulsions. From age 28 (21%) followed by seborrhoeic keratosis in 16 (14%), actinic years intermittent treatment with oral fluconazole has helped keratosis in 15 (13%), melanocytic naevus in 13 (12%), warts her candidiasis. Patient 2 has two children, neither of whom in six (5%), Bowen’s disease in five (4%), squamous cell car- have CMCC or associated diseases. Patient 3 developed persist- cinoma (SCC) in six (5%), dermatofibroma in three (3%), ent oral candidiasis at the age of 3 years. From the age of 23 eczema in three (3%), psoriasis in two (2%) and miscellan- years he has been treated with long-term itraconazole, which eous conditions in 20 (18%) cases. A history of trauma, either improved his oropharyngeal candidiasis, and topical miconaz- simultaneous or preceding the onset of the was ole gel for angular cheilitis. At the age of 5 years he developed present in 11 (10%) cases. This comprised five patients with primary hypothyroidism. His 3-year-old son has recently been BCC, four patients with miscellaneous conditions and one diagnosed with oral candidiasis. Patients 2 and 3 tested nega- patient each with and dermatofibroma. A vol- tive for the two common AIRE1 mutations, found in 80% of unteered history of trauma was common among patients with patients with autoimmune polyendocrinopathy–candidiasis– BCC, being present in 21% of cases. The type of trauma was ectodermal dystrophy syndrome (APECED). Apart from pri- mechanical in all and blunt in all but one case. It occurred mary hypothyroidism affecting the father and son (patients 1 simultaneously with onset in four (80%) and preceded onset and 3), there is no endocrinopathy or organ-specific autoim- by less than a month in one (20%) patient. No case of SCC mune disease in the reported family. The association of CMCC gave a history of trauma. Trauma has been implicated in the with primary hypothyroidism, an autosomal dominant pattern past as an aetiological factor in the development of BCC/SCC of inheritance, without evidence of common AIRE1 mutations, and postulated to act either by promoting local actinic damage endocrinopathies or autoimmune disease, is against the diag- or as a cocarcinogen (Rustin MHA, Chambers TJ, Munro DD. nosis of recessively inherited APECED. The presentation is in Post-traumatic basal cell carcinomas. Clin Exp Dermatol 1984; 9: keeping with the distinct autosomal dominantly inherited 379–83). Our results do not confirm this aetiological role, as CMCC syndrome associated with primary hypothyroidism the history of trauma and the onset of the lesion were mostly (Coleman R, Hay RJ. Chronic mucocutaneous candidosis asso- simultaneous. Our survey rather suggests that trauma brings to ciated with hypothyroidism: a distinct syndrome? Br J Dermatol attention a previously unnoticed lesion. This is particularly 1997; 136: 24–9). More recently the gene defect for this syn- likely in insidiously and slowly progressing conditions like drome has been mapped to chromosome 2p (MIM 606415) BCC. (Atkinson TP, Scha¨ffer AA, Grimbacher B et al. An immune defect causing dominant chronic mucocutaneous candidiasis and thyroid disease maps to chromosome 2p in a single fam- P-60 ily. Am J Hum Genet 2001; 69: 791–803). We report this family Adjuvant treatment of extramammary Paget’s to increase awareness of this rare variant of CMCC and disease using 5% imiquimod cream describe oesophageal carcinoma arising as a complication of V. Brown, N.P.J. Walker and V.A. Venning CMCC for the first time. Department of Dermatology, Churchill Hopsital, Oxford, U.K. Extramammary Paget’s disease (EMPD) is a rare cutaneous tumour that usually involves the anogenital region. It can be P-59 difficult to manage by surgery alone since it may have a Lumps and bumps: the role of trauma multifocal origin, often affects an extensive area at diagnosis S. Madhogaria, M. Khan and A. Ilchyshyn and typically has indistinct clinical margins that extend beyond University Hospital, Coventry and Warwickshire, Coventry, U.K. the obviously affected tissue. Imiquimod, a topical immune Trauma has been commonly implicated as a precipitating or response modifier, is a promising new topical treatment for aggravating factor in several dermatological conditions, par- EMPD. It acts through local augmentation of the innate and ticularly skin malignancies. We performed a survey to evaluate acquired immune system, stimulating cytokine production and the history of trauma and its temporal association to different apoptosis. We have recently used 5% imiquimod cream

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(Aldara, 3M Health Care, Loughborough, U.K.) as adjuvant skin biopsy showed a normal epidermis with a nodular peri- treatment in two patients with EMPD. A 76-year-old woman adnexal dermal infiltrate extending to the subcutis. This was presented with a 3-year history of non-itchy perianal skin composed of lymphocytes, histiocytes and large atypical multi- lesions. Examination revealed two verrucous plaques within nucleate cells which were positive for CD45, CD20, CD79, background xerotic, erythematous perianal skin. Skin biopsy CD30 and MIB1, but EMA and ALK negative. In addition, the confirmed the diagnosis of EMPD, with a focus of invasive large B cells were all positive for Epstein–Barr virus (EBV) adenocarcinoma. Subsequent investigations failed to detect an latent membrane protein. The findings suggest a histological internal malignancy. The verrucous areas were excised with diagnosis of lymphomatoid granulomatosis grade 3. Staging primary closure, showing adequate histological margins of the investigations including a computed tomography scan of the invasive disease but involvement of peripheral margins with chest, abdomen, pelvis and brain, bone marrow aspirate and EMPD. Treatment was therefore commenced with imiquimod trephine and screening bloods did not demonstrate extracuta- once daily on weekdays for 12 weeks. The brisk inflammatory neous involvement. EBV serology showed evidence of prior response generated, was well tolerated and settled soon after infection. Discontinuation of the anti-TNF-a therapy led to stopping treatment. At 6-week follow-up, a skin biopsy from initial clinical and histological resolution of the lesions. How- a residual area of roughness showed no histological evidence ever his arthritis deteriorated and local relapse has since of EMPD. There have been no signs of recurrence to date, 4 occurred. Options for management now include antiviral ther- months after stopping imiquimod. A 57-year-old man with a apy with ganciclovir and/or rituximab with discontinuation of 10-year history of perianal soreness and pruritus was diag- methotrexate. EVB associated lymphoproliferative disorder nosed with extensive perianal EMPD following a skin biopsy. (EBV LPD) is well described in autoimmune disease, primary Appropriate investigations excluded an associated internal ma- and iatrogenic immunosuppression (in particular with metho- lignancy. Our patient wished to avoid the radical surgery that trexate and nucleoside analogues). Primary cutaneous manifes- would be necessary to clear his disease and was instead treated tations of EBV LPD are uncommon. Lymphomatoid with imiquimod (three times a week for 4 weeks then five granulomatosis (LyG) is a rare extranodal form of EBV LPD times a week for 6 weeks). After the severe local inflammation which is characterized histologically by an angiocentric, angio- provoked by the treatment had abated, only half the affected destructive lymphohistiocytic infiltrate composed of a reactive area had cleared clinically. He was therefore commenced on polyclonal T cell population and large atypical EBV+ B cells. It triple therapy with 5-fluorouracil, 0Æ1% tretinoin and imiqui- is classically a multisystem disease with pulmonary, renal, CNS mod, with each agent applied once daily for 2 weeks. Minimal and skin involvement in 25–50% of cases. To our knowledge further improvement was noted. In an attempt to reduce the there are only three reported cases of isolated cutaneous dis- extent of future surgery, he is currently being considered for ease in the literature. The incidence of lymphoma in associ- radiotherapy. Our two cases add to the limited published data ation with anti-TNF-a therapy is rising. There are no previous on the use of imiquimod in the management of EMPD. They reports of LyG in the setting of infliximab or other anti-TNF-a illustrate both the variable clinical response and the versatility therapies. Our patient had been on methotrexate for a number of imiquimod for either preoperatively reducing the extent of of years; however, as TNF-a has a key role in promoting cyto- disease or for postoperative treatment of residual tumour. Our toxic T cell responses to EBV-induced B cell proliferation, it is patients used imiquimod in conjunction with other treatment possible that infliximab therapy has also played an aetiological modalities, but there are anecdotal reports of imiquimod mo- role. notherapy producing complete clearance of EMPD. Imiquimod has potential use in a variety of circumstances in the manage- ment of EMPD. P-62 Can the two-week skin cancer referral proforma be refined to improve sensitivity and specificity of P-61 referrals? Epstein–Barr virus positive lymphoproliferative V. Madan, T. Sanders and N.H. Cox disorder presenting as isolated cutaneous Cumberland Infirmary, Carlisle, U.K. lymphomatoid granulomatosis in a patient treated Previous research has shown that the two week rule (TWR) with anti-TNF-a therapy and methotrexate for skin cancer is not ideal. In a national study a median of N. Desai, R. Stitson, S. Patel and C.A. Holden 10% of referrals were relevant tumours, and only 50% of St Helier Hospital, Carshalton, U.K. tumours overall (12% of squamous cell carcinomas, SCC) A 78-year-old man presented with a four-week history of a were referred by this mechanism. We have audited local TWR rapidly enlarging nodular eruption on the right knee. He had skin cancer data for the last 18 months (506 patients) and had seronegative rheumatoid arthritis, treated with methotrex- have used the melanomas from this group, and a larger group ate for nine years, plus anti-TNF-a therapy (infliximab) for of 181 SCC, to evaluate possible manipulations to the standard three years. Examination revealed scattered ulcerated nodules form that might increase pick-up of relevant tumours. Pig- and indurated plaques over the right medial knee, extending mented lesions comprised 75% of referrals, of which 30 of to the thigh. There was no associated lymphadenopathy. A 381 (8%) were melanoma; for referrals as ?SCC, 22 of 125

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(18%) were confirmed. However, only 8% of 181 SCC were 2005 and 2006. The hospital notes of the melanoma cases referred by TWR. For SCC, over 90% were referred as enlar- were reviewed with specific reference to changes made to the ging indurated lesions and less than 10% on the basis of histopathology report and planned clinical or surgical manage- immunosuppression or primary care biopsy. Thus any change ment. Ninety-six cases of melanoma occurring in 95 individu- that would alter referral patterns would need to be in the area als were reviewed. In three cases the diagnosis was changed, of lesion identification. A possible manipulation is to alter the in one from a squamous cell carcinoma to a melanoma, in recommended 1 cm size guidance within the TWR back- another case from a dysplastic naevus to melanoma in situ and ground documentation. However, of 82 SCC where we had in a further case from invasive melanoma to a Spitz naevus. In data on measured size, the 1 cm cut-off picked up 89%. A 16 (17%) cases of melanoma the Breslow thickness was size of 7 mm would have achieved identification of over 90% revised (five decreased and 11 increased). Ulceration, previ- but our data suggested that size is often estimated anyhow, ously unreported, was noted in two cases. The histological and this change would be very unlikely to do much other revision led to a change in melanoma staging (Balch CM, Buz- than to increase non-SCC referrals. For melanoma, the identifi- aid AC, Soong SJ et al. Final version of the American Joint cation system uses the 7-point checklist. Options to improve Committee on Cancer Staging System for cutaneous mela- accuracy might include defining a specific number of points noma. J Clin Oncol 2001; 19:3 635–48) in 13 (14%) cases. to tick on the TWR proforma, or using the revised checklist Alteration in Breslow thickness in 16 patients changed recom- with weighted scoring (score up to 10 instead of 7). How- mended surgical management (Roberts DLL, Anstey AV, Bar- ever, neither of these methods had any diagnostic discriminat- low RJ et al. U.K. guidelines for the management of cutaneous ory value. The mean score for 30 melanomas was 3Æ06 of melanoma. Br J Dermatol 2002; 146: 7–17) in nine (9%) cases. total score 7 and for 351 non melanomas was 2Æ82 of total Following MDT histopathological review, staging in three score 7 (nonsignificant, P =0Æ8); corresponding scores using patients was upgraded, and in one case the diagnosis was the revised checklist were 4Æ70 of total score 10 and 4Æ45 of altered from SCC to melanoma, such that all four patients total score 10 (nonsignificant, P =0Æ74). Additionally, no in- were offered entry into melanoma clinical research trials. dividual tickbox had any apparent link with the diagnosis of Although skin cancer MDT review meetings are recommended melanoma vs. nonmelanoma, and a series of sensitivity and by NICE, there are no studies evaluating their effectiveness. specificity evaluations corresponding to each score showed Our study shows that they are very valuable, as melanoma that no score on either the 7- or the 10-point scale had an management decisions in relation to surgery, prognosis and acceptable combination of sensitivity and specificity. For recruitment to trials, were altered in 16 (17%) of these cases. example, the traditional score of 3 on the 7 point checklist had sensitivity of 67% and specificity of 37%, a score of 4 on the revised checklist (maximum score 10) had the same 67% P-64 sensitivity with specificity of 31%. It appears that ticking a sin- Improved efficacy of efalizumab as adjunctive ther- gle box to trigger referral overrides any value of further apy in moderate to severe psoriasis: short term and options. The TWR form therefore needs no more options than maintenance safety data in 10 patients ?SCC or ?melanoma; we suggest that a simpler form and more R. Mak and R. Cerio education would be most valuable. Dermatology Department, Royal London Hospital, Whitechapel, London, U.K. Moderate to severe psoriasis constitutes about 40–50% of all patients with psoriasis and is associated with significant mor- P-63 bidity. All standard systemic therapies are associated with The value of a skin cancer multidisciplinary team potential long-term toxicity with a risk of treatment-resistant meeting disease. Biologics have emerged as potentially valuable alterna- R. Ramesh, K. Batta, F.M. Tatnall, P. Nathan,* tives. To date, most published data describes their use as A. Rubin and M.E. Murdoch monotherapy. However, it is unusual for a single agent to be West Herts Hospital NHS Trust, Watford, Herts, U.K. and *Mount Vernon universally effective. In contrast to most licensed biologics, Cancer Centre, Northwood, Middlesex, U.K. efalizumab is a humanized monoclonal antibody to leucocyte The National Institute for Health and Clinical Excellence function associated antigen (LFA)-1 and appears to have a (NICE) guidance on cancer services outlined in February good safety profile but reduced efficacy compared with 2006, recommends that all patients with melanoma and squa- tumour necrosis factor (TNF)-a antagonists. It inhibits the mous cell carcinoma should be referred for a multidisciplinary migration of T cells into psoriatic skin. It has recently been team meeting (MDT) review. Watford General Hospital has given both a license and NICE approval in the U.K. for moder- had a Skin Cancer MDT in place since 2002. The team com- ate to severe psoriasis. To our knowledge, little has been pub- prises dermatologists, an oncologist, histopathologists and a lished on its use as adjunctive therapy with standard systemic plastic surgeon. Here we review how this process influences agents. We conducted a pilot study of 10 patients where patient management, particularly in relation to melanoma. The efalizumab was used in conjunction with other recognized minutes of MDT meetings were examined retrospectively to systemic treatment. Seven male and three female patients, aged identify all melanoma cases referred for MDT review between between 34 and 60 years old with an average age of 46 years

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 51 were recruited. The average duration of psoriasis was 20 years ANA at 1 in 640 with a homogenous pattern. A subsequent and all patients had been treated with oral drugs. Their mean liver biopsy confirmed features of autoimmune hepatitis. The baseline Psoriasis Area and Severity Index (PASI) score was 19; patient was commenced on oral prednisone and azathioprine mean Dermatology Quality of Life Index (DQLI) was 17 out was added to this at a later date. The patient’s LFTs improved, of 30 and Global Evaluation Score (GES) ranged from moder- as did the psoriasis. As the dose of prednisone was reduced ate to severe. All patients had received phototherapy and the skin flared and alternative biologic agents, such as extensive topical treatment. Moreover, two patients were efalizumab are being considered. Infliximab is a humanized on methotrexate, one on leflunomide, two on a hydroxyurea anti-TNF-a antibody licensed for use in patients with psoriasis. and etretinate combination, two on ciclosporin and one on The potential adverse effects of infliximab are well known and mycophenolate mofetil. Two patients were dependent on include reactivation of tuberculosis, opportunistic infections, phototherapy (including PUVA). Following treatment guide- demyelination and acute hypersensitivity reactions. Infliximab- lines, all patients were started on efalizumab at a loading dose associated hepatitis has been previously reported, with the of 0Æ7mgkg–1 week–1 before receiving 1 mg kg–1 week–1 for majority of these cases resulting from reactivation of chronic 12 weeks minimum with no wash-out period. Benefit was hepatitis B infection. A further report suggested that infliximab observed for all 10 at ‡ 4 weeks. Systemic therapies were sub- could be the direct cause of hepatitis in the absence of a sequently significantly reduced after 3 months. A significant detectable aetiology or concurrent pathology. Infliximab-asso- improvement was seen in PASI score by an average of 42% ciated autoimmune hepatitis is a very rare event. To our (i.e. 11) and maintained for a mean of 26 weeks. Mean DQLI knowledge it has never previously been reported in a patient improved by an average of 24% (13 out of 30) and GES now with psoriasis. Two previous reported cases have been in ranged from mild to moderate. Two patients required no fur- patients with rheumatoid and psoriatic arthritis. In these cases ther topical steroid despite suberythroderma. Moreover, four hepatitis developed many months into their treatment, e.g. of six patients on oral therapy were able to reduce and then after the 12th infusion (Tobon GJ, Canas C, Jaller JJ. Serious stop their systemic therapy before 12 weeks. The side-effects liver disease induced by infliximab. Clin Rheumatol 2006; Epub of efalizumab including temporary flu-like symptoms and ahead of print). In our case this occurred after just the 3rd headache were only reported in two patients. Universally, infusion. This highlights the need for strict monitoring of blood monitoring, especially of platelets, remained normal. patients even in the early stages of infliximab therapy. Abnor- We concluded that efalizumab used in combination is an ef- malities of liver function tests should alert clinicians to the fective and safe adjunctive treatment in moderate to severe search for the underlying aetiology with checking of autoanti- psoriasis and the benefit of the combination appears better body status to exclude infliximab-associated autoimmune than that of monotherapy. A large, double-blinded random- hepatitis. ized-controlled trial should be carried out to confirm its syner- gistic effect. P-66 The association between age of onset of psoriasis P-65 and comorbidities Infliximab-associated autoimmune hepatitis in a G. Hughes, E. Kleyn and C.E.M. Griffiths patient with palmoplantar pustular psoriasis The University of Manchester, The Dermatology Centre, Manchester, U.K. D. Fairhurst and R. Sheehan-Dare There is increasing evidence that psoriasis is associated with The General Infirmary at Leeds, Leeds, U.K. features of the metabolic syndrome, namely, hyperlipidaemia, A 22-year-old lady presented with a 5-month history of hypertension, obesity and diabetes mellitus. The underlying palmoplantar pustular psoriasis to both hands and feet. The pathomechanisms of this association have not been delineated condition was resistant to conventional therapy including top- although a chronic inflammatory state, akin to rheumatoid ical therapy, oral PUVA, ciclosporin up to a dose of arthritis, is implicated. We interrogated an electronic database 4Æ5mgkg–1 and methotrexate up to a dose of 20 mg weekly. to determine the association between early-onset psoriasis Due to a poor response to the above therapies the patient was (EOP; type 1; presenting before age 40 years) and late-onset considered for infliximab. Baseline liver function tests (LFT) psoriasis (LOP; type 2; presenting after age 40 years) with were normal and hepatitis B and C serology were negative. hyperlipidaemia, hypertension and body mass index (BMI). Antinuclear antibodies were found to be only weakly positive Data acquired from 973 new patients attending a psoriasis at 1 in 40. Infliximab monotherapy was commenced at a dose specialist clinic over a 6-year period (2001-6) were analysed of 5 mg kg–1, given at week 0, 2 and 6. A dramatic clinical using multivariate logistic regression. The data comprised 793 improvement was noted after the first two infusions. Unfortu- (82%) patients with EOP and 180 (18%) with LOP. In EOP, nately the palmoplantar pustulosis deteriorated after the third the average age of onset and disease duration was similar for infusion. Two weeks after the 3rd infliximab infusion, the males [n = 438 (54%); disease onset: 19 ± 9Æ2 years; disease patient was found to have an elevated ALT level at 168 i.u. L–1. duration: 18 ± 11Æ9 years] and females [n = 355 (46%)]; dis- Over the next 28 days this level increased to 1663 i.u. L–1.A ease onset: 17 ± 9Æ8 years; disease duration: 19 ± 14Æ8 years]. hepatitis screen was negative except for a strongly positive Similarly, LOP males [n = 95 (53%); disease onset: 49 ± 8Æ1

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 52 Bristol Cup Posters years; disease duration: 8Æ7 ± 7 years] and females [n =85 form of treatment used in primary care [44 of 57 (77%)] (47%); disease onset: 52 ± 9Æ8 years; disease duration: 10 ± prior to hospital referral. Other treatments tried in primary 9Æ5 years] had analogous data in terms of age of onset and care included vitamin D analogues [14 of 57 (24%)], coal tar disease duration. A family history of first degree relatives with [9 of 57 (16%)] and salicylic acid [5 of 57 (9%)]. In second- psoriasis was reported by 52% of EOP patients in contrast to ary care, response rates to treatments were: coal tar [5 of 7 29% of LOP patients. After controlling for other factors such (71%)], systemic PUVA [1 of 2 (50%)], acitretin + topical as age, LOP appears significantly associated with an increase in PUVA [1 of 2 (50%)], ciclosporin [1 of 2 (50%)], acitretin the odds of developing hyperlipidaemia when compared with (1 had etretinate) [6 of 13 (46%)], salicylic acid [3 of 7 EOP [odds ratio (OR) = 6Æ35, 95% confidence interval (CI): (43%)], topical corticosteroid + coal tar + salicylic acid [3 of 1Æ2–34Æ2, P =0Æ03]. The subgroup of psoriasis did not signifi- 7 (43%)], coal tar + salicylic acid [2 of 5 (40%)], topical cantly increase the risk of a high BMI; however, females (ideal PUVA [8 of 21 (38%)], methotrexate [3 of 9 (33%)], tetracy- range: 19Æ1–25Æ8; EOP: 27 ± 6Æ9; LOP: 29Æ8±6Æ8) were clines [2 of 6 (33%)], topical corticosteroid [5 of 26 (19%)], more likely to have a BMI above normal range than males topical vitamin D analogue [3 of 17 (18%)], topical cortico- (ideal range: 20Æ7–26Æ4; EOP: 27 ± 5Æ6; LOP: 28 ± 6). The steroid + salicylic acid [2 of 14 (14%)], topical corticosteroid type of psoriasis did not increase the risk of having hyperten- + occlusion [1 of 8 (13%)], and topical vitamin D analogue sion (OR = 1Æ37, 95% CI: 0Æ59–3Æ18, P =0Æ46); however, + corticosteroid [3 of 28 (11%)]. Two patients given acitretin we observed that systemic medications (OR = 2Æ08, 95% CI: in combination with systemic PUVA, one given methotrexate 1Æ08–4Æ01, P =0Æ03) appeared to increase the odds of devel- along with ciclosporin, and one given fumaric acid esters had oping hypertension across both groups. These data suggest a satisfactory response to treatment. In conclusion, combin- that subgrouping of type of psoriasis according to age of onset ations of coal tar and salicylic acid are safe and effective in the may be important in calculating the odds of developing hyp- management of PPP, and increased use of these in primary erlipidaemia but not that of hypertension. Systemic medica- care may reduce referral rates. Current use of these agents in tions may confer a risk of hypertension in both EOP and LOP. primary care is low, mostly because sourcing from commu- Further study of the association between psoriasis and meta- nity pharmacies is difficult, and this should be improved. In bolic syndrome is required to ensure timely monitoring of secondary care, a variety of agents need to be tried due to the risk factors and treatment of comorbid disease. heterogeneous response of this challenging condition.

P-67 P-68 A regional audit of the management of palmoplantar New onset of palmoplantar pustulosis under tumour pustulosis with recommendations on treatment in necrosis factor a blockers primary care H. Bauer, P. Helliwell, K. London and A.L. Wright A. Bharati and R.A.G. Parslew Bradford Teaching Hospitals NHS Foundation Trust, Bradford, U.K. Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, We report the case of a 66-year-old male patient with a 20- U.K. year history of rheumatoid arthritis who developed palmo- Palmoplantar pustulosis (PPP) is a chronic inflammatory skin plantar pustulosis after ten pulses of infliximab 200 mg i.v. in condition that presents as crops of sterile pustules on the combination with leflunomide. There was no past medical his- palms and soles. Although many different treatments are used tory or family history of psoriasis. The patient had not suf- none is generally accepted as being reliably effective. A region- fered a preceding bacterial infection or febrile illness before al audit was performed with the aims of studying the manage- developing the skin lesions. Infliximab and leflunomide were ment of PPP. The methods used were (i) a questionnaire subsequently discontinued and replaced by adalimumab 40 mg survey of 38 dermatology practitioners and (ii) a case-note s.c. every other week, without improvement of the skin review of 57 patients with PPP. Completed questionnaires lesions after 5 months. Both tumour necrosis factor (TNF)-a were received from 34 (89%) of the 38 practitioners of differ- blockers used showed satisfactory reduction of arthritis symp- ent grades (16 consultants, two associate specialists, 11 spe- toms and inflammatory markers. Bacteriological and fungal in- cialist registrars, three staff grade physicians and two clinical vestigations from skin scrapings and pustule contents were assistants). Treatments considered to be appropriate for first repeatedly negative. The patient was started on an eight-week line use in primary care were topical vitamin D analogues [32 course of hand and foot photochemotherapy (PUVA) com- of 34 (94%)], corticosteroids [31 of 34 (91%)], coal tar [20 bined with topical clobetasol propionate 0Æ05%, neomycin sul- of 34 (59%)] and occlusion [16 of 34 (47%)]. The 57 phate 0Æ5% and nystatin 105 Ug–1 cream twice daily with patients with PPP consisted of 44 females (77%) and 13 males only marginal improvement. This was discontinued after a (23%). The mean age of onset of PPP was 49 years (range cumulative dosage of 18 J cm–2 ultraviolet (UV)A radiation. 21–79). Of those whose smoking history was available (35 of Treatment with acitretin 20 mg daily by mouth was com- 44), 80% were smokers. One-third of all patients had psoriasis menced, which showed significant improvement within four affecting nonpalmoplantar skin, while psoriatic arthritis was weeks, however, the patient relapsed after a further three present in six (11%). Topical corticosteroid was the main months. Recently a number of cases with a new onset of pre-

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 53 dominantly pustular psoriasis have been reported in patients make this active substance an important tool in the manage- with rheumatoid arthritis and ankylosing spondylitis receiving ment of psoriasis. treatment with TNF-a blockers, which seems paradoxical (Kary S, Worm M, Audring H et al. New onset or exacerbation of psoriatic skin lesions in patients with definite rheumatoid arthritis receiving tumour necrosis factor alpha antagonists. P-70 Ann Rheum Dis 2006; 65: 405–7). Some authors have suggested Quantiferon gold testing for tuberculosis screening that palmoplantar pustulosis might represent keratoderma in psoriasis patients prior to anti-tumour necrosis blenorrhagicum, particularly if it includes onycholysis and factor therapy subungual keratosis, as was the case in our patient. This prob- Y. Raste, N. Desai, N. Cooke and C.C. Harland lem might be related to persistence or reactivation of infec- St Helier Hospital, Carshalton, Surrey, U.K. tious organisms or superantigens like Chlamydia trachomatis or NICE guidelines published in July 2006 advise etanercept, a Yersinia under immune suppression by TNF-a blockers (Carter tumour necrosis factor (TNF) antagonist, as a treatment for JD. Tumour necrosis factor inhibition causing psoriasis? A severe plaque psoriasis in patients who have not responded to more plausible explanation exists. (Letter) Ann Rheum Dis 2006; other therapies. Patients embarking on any immunosuppres- 65: 1680). Other authors have discussed an imbalance of sive treatment require screening for tuberculosis (TB). This TNF-a and interferon-a promoting an autoimmune response. has traditionally consisted of Mantoux skin testing with risk These-side effects are not specific to one single type of TNF stratification for exposure from the history. The problem with antagonist and change of anti-TNF agent does not necessarily skin testing and screening is cross-reactivity with the BCG vac- improve skin symptoms. Further investigations are required cine, which often gives false-positive results. This may lead to into these phenomena, which are currently poorly under- unnecessary chemoprophylaxis, not only delaying the start of stood. immunosuppressive therapy but also carrying with it possibly dangerous side-effects. There are also practical difficulties in reading the Mantoux on patients with severe psoriasis who P-69 have widely affected skin and the cost implications of unnec- Ciclosporin 1% liposomal gel in limited plaque essary treatment for TB are considerable. The Quantiferon gold psoriasis: a prospective randomized double-blind blood test (Statens Serum Institut, Copenhagen, Denmark) has placebo-controlled study in stable plaque psoriasis recently become available and is thought to be more sensitive T. Narang, S. Dogra, I. Kaur, O.P. Katare and and specific than Mantoux testing. It is an in vitro test based on R. Khurana interferon c release from leucocytes in response to specific TB Postgraduate Institute of Medical Education and Research, Chandigarh, India antigens, which only occurs in patients infected with Mycobact- Drug delivery systems using colloidal particulate carriers such erium tuberculosis. Importantly, these antigens are not present in as liposomes have distinct advantages over conventional dos- BCG so there is no crossreactivity in patients who have previ- age forms because the particles can act as drug-containing res- ously been vaccinated. Practically, the test is very easy to carry ervoirs. Ciclosporin is one of the important drugs for the out and a result is available within 3 days. We report a study management of psoriasis but because of adverse events and of nine patients suffering from moderate to severe psoriasis the need for frequent monitoring its use is limited in stable (ranging in age from 27 to 56 years, two female, seven plaque psoriasis. We prepared a novel, aqueous gel-based, male), considered suitable for etanercept. The Quantiferon liposome-entrapped formulation of ciclosporin. The present gold test was used to screen these patients and was compared study was planned to evaluate the efficacy and tolerability of to Mantoux testing. Six Quantiferon gold tests were negative 1% ciclosporin liposomal gel administered once daily for 8 and six were indeterminate. Of the negative tests, all had weeks in the treatment of stable plaque psoriasis. This was a negative Mantoux tests except one male patient who had a prospective, right-left randomized study of 8 weeks duration. Mantoux test of 20 mm diameter – he had previously been The study consisted of 20 patients, having almost bilateral vaccinated with BCG but had no risk factors for exposure. symmetrical stable psoriasis plaques over the limbs (measuring Tuberculosis chemoprophylaxis was avoided on the basis of less than 100 cm2). Lesions treated with the liposomal ciclosp- his negative Quantiferon test. The cost of each test was £58. orin gel formulation showed marked improvement in com- This compares with the projected cost of unnecessary chemo- parison to placebo gel. The reduction in erythema, scaling and prophylaxis for 2 months of approximately £750. This feasi- induration (ESI) score was significantly more at both 4 and 8 bility study confirms that the Quantiferon test is useful for weeks on the side treated with 1% ciclosporin liposomal gel tuberculosis screening prior to anti-TNF therapy, potentially (P <0Æ05). In twelve patients, there was total clearance of saving patients from unnecessary chemoprophylaxis for TB. lesions. Four patients showed more than 50% improvement. There are also cost saving implications as multiple hospital vis- Few patients experienced mild adverse events. No clinically its for the Mantoux method are avoided. The Quantiferon gold significant changes in blood or other lab parameters were test is currently licensed in the United States for screening for seen. The efficacy and tolerability of liposomal ciclosporin gel tuberculosis. Its use in the U.K. needs to be further evaluated.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 54 Bristol Cup Posters

situation. The aim was to determine the efficacy and safety of P-71 Initial validation and refinement of the Psoriasis 5% clobetasol propionate in a lacquer vehicle in nail psoriasis. Family Index Ten patients with both nail bed and matrix psoriasis were A.M. Eghlileb, M.K.A Basra and A.Y. Finlay included in the study. They were treated with a nail lacquer Dermatology Department, Cardiff University, Cardiff, U.K. containing 5% clobetasol propionate that was applied once Skin diseases are known to have an extensive impact on the daily for 16 weeks. After discontinuation of therapy, follow lives of patients’ families (Basra MK, Sue-Ho R, Finlay AY. up visits were performed at 4 and 8 weeks. Within 4 weeks The Family Dermatology Life Quality Index: measuring the of therapy there was a reduction of all the nail alterations in secondary impact of skin disease. Br J Dermatol 2007; 156: all the patients; two of them were completely free from nail 528–38). The purpose of this study was initially to validate lesions after 12 weeks. The nail parameters that responded the Psoriasis Family Index (PFI) and then refine and shorten best to therapy were onycholysis, pitting and salmon patches. it. The PFI is a new instrument being developed to measure Subungual hyperkeratosis and splinter haemorrhages on the the secondary impact of psoriasis on the quality of life (QoL) other hand had moderate and poor improvement, respectively. of families and partners of affected individuals (Eghlileb AM, The treatment was well tolerated in all of the patients and Davies EEG, Finlay AY. The secondary impact of psoriasis on there were no local (i.e. atrophy and secondary infection) or the family: the Psoriasis Family Index. Br J Dermatol 2006; 155 systemic secondary effects. The formulation containing 5% (Suppl. 1): 31. Abstract). Fifty-one adult family members or clobetasol propionate is a safe, effective and cosmetically partners of patients with psoriasis completed the 20 item ver- acceptable treatment for nail psoriasis. sion of the PFI (PFI-20). The total PFI-20 score ranges from 0 to 60; the higher the score the greater the impairment of QoL. Subjects were excluded if they suffered from any other P-73 significant disease. Factor analysis and a test for internal con- Three years’ experience of the management of sistency were conducted to assess the psychometric properties patients with delusional parasitosis in a joint of the PFI-20. Of the 51 recruited participants 12 were family dermatology and liaison psychiatry clinic members and 39 were partners (M = 11, F = 40, mean age R. Healy, S. Dhoat, R. Taylor and A.P. Bewley = 48 years). The mean duration of patients’ disease was 18Æ6 Barts and the London NHS Trust, London, U.K. years. The mean PFI-20 score was 23 (SD = 12Æ7, range 4– Delusional parasitosis (DP) is a well recognized uncommon 54). The score was higher for female subjects (mean = 24Æ8, condition which patients often present to a dermatology cli- SD = 12Æ0) than male (mean = 16Æ3, SD = 13Æ4, P =0Æ05). nic. Patients with DP hold a fixed belief that they are infested Although the score was higher for subjects whose relatives with organisms such as unicellular parasites, bacteria, viruses were receiving topical treatment (mean = 24Æ2, SD = 12Æ6) or insects. Patients with DP are notoriously difficult to man- compared with those receiving systemic therapy (mean = age. When referred to a purely psychiatric clinic, patients with 19Æ9, SD = 12Æ7), this was not significant (P =0Æ25). Seven DP often fail to attend their appointments. Also, it is very dif- factors were extracted using principal component analysis ficult to manage patients with DP in a standard dermatology which explained 67% of total variance. However, the internal clinic as the patients may take considerable time, and other consistency of the scale using Cronbach’s a was only 0Æ64. resources, to engage in therapeutic management. For these Therefore items with low item-total correlation (< 0Æ20) were reasons we established a joint dermatology and liaison psychi- eliminated while other items were rewritten to reduce ambi- atry clinic in 2002. Patients are referred to the clinic from our guity and make them more clear. A shorter version of the PFI own catchment area, from surrounding secondary care hospi- is now being developed as a multidimensional measure con- tals and from further afield. We have audited our practice for sistent with the concept of QoL. Further validation of the three years (Jan 2004–Jan 2007) and include our findings in instrument is required to allow it to be used for assessing QoL this report. Over the three year period we have managed 21 in family members and partners of patients with psoriasis. patients with DP (13 females, eight males; mean age 52 years; age range 33–74 years). Seventeen patients were tertiary refer- rals from surrounding secondary care dermatology units. Patients had suffered from their disease for an average of 5Æ5 P-72 years (range: 1–12Æ5 years). Management of patients was usu- Clobetasol nail lacquer: a safe, effective and ally with atypical antipsychotic medication; risperidone was cosmetically acceptable treatment for nail psoriasis used to treat 10 patients and olanzapine two patients. Three T. Narang, S. Dogra, I. Kaur, O.P. Katare and patients responded to simple counselling and required no sys- A. Bhatia temic medication, six patients were treated solely with antide- Postgraduate Institute of Medical Education and Research, Chandigarh, India pressant medication including amitriptyline, fluoxetine, Compared with the advances in the past years for the treat- doxepin and citalopram. The dose of all medications used was ment of skin psoriasis, therapy of nail psoriasis has been far variable. Some patients required both antipsychotic and anti- from satisfactory. Several topical therapies have been employed depressant medication. Five patients have failed long-term fol- with poor results because drug penetration is limited in this low-up (two patients are known, from telephone review, to

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 55 be well). Three patients improved without medication, two (BOLD) contrast were acquired continuously for 6Æ8 minutes patients continue to attend our clinic regularly, but, to date, during the presentation of facial expressions using a Philips 3 have refused to take psychotropic medication (these patients, Tesla Achieva MR (Royal Philips Electronics, Eindhoven, the however, continue to engage with clinic doctors, and have Netherlands) scanner. Average images of disgust-neutral and shown some response to emollients). Thirteen patients (in- fear-neutral were created for each subject and then analysed cluding the two who have been followed up by telephone) using a random effects ANOVA in Statistical Parametric Mapping have responded well to medication (skin lesions have (SPM5, Wellcome Department of Imaging Neuroscience, Uni- improved or resolved, and the delusion is either no longer versity College, London, U.K.) at a threshold of Punc <0Æ001. held or is less fixed). All patients have had careful treatment Processing of disgust-neutral facial expressions resulted in a of their cutaneous disease with emollients, antibiotics, topical significant reduction of BOLD signal bilaterally in the insula in steroids and antipruritic medications. One patient, to date, has subjects with psoriasis compared to controls. In response to relapsed after stopping systemic medication. The families of fearful faces, both patients and controls demonstrated an two patients with DP have also had symptoms (folie a` deux). increase in BOLD signal bilaterally in the amygdala and tem- Treatment of the index patient with DP has resolved the poral pole but there was no significant difference between the symptoms in these families. In conclusion, the management of groups in terms of this response. The results demonstrate, for patients with DP in standard dermatology and/or standard the first time, evidence for a differentiation between insula psychiatric clinics is difficult. We present the successful man- responses to disgust in patients with psoriasis as compared to agement of many patients with DP in a joint dermatology/ healthy volunteers. Our interpretation of these data is that fre- liaison psychiatry clinic. quent exposure of patients to disgusted facial expressions causes attenuation of insula activation. Moreover, as shown by lack of difference in BOLD response between the groups to P-74 fearful facial expressions, the differential response appears spe- Neural correlates of processing facial expressions of cific to perception of disgust and not aversive facial expres- disgust in patients with psoriasis: a functional brain sions in general. This study was funded by an unrestricted imaging study educational grant from Serono, Zug, Switzerland. E. Kleyn, A. Ross, S. McKie,* L. Gregory, D. Montaldi, C.L. Isaac, H. Richards, D. Fortune and C.E.M. Griffiths P-75 The University of Manchester, The Dermatology Centre, Manchester, U.K.; Poor prognosis in delusion of parasitosis: lessons *The University of Manchester, Division of Psychiatry, Manchester, U.K.; learnt The University of Manchester, School of Psychological Sciences, Manchester, K. Ahmad and B.C.P. Ramsay U.K. and The University of Sheffield, Sheffield, U.K. Mid-Western Regional Hospital, Limerick, Ireland Cerebral processing of facial expressions plays a key role in Delusion of parasitosis (DP) is a difficult psychiatric condition our emotional and social behaviour. Patients with psoriasis and patients usually present to dermatology clinic looking for may experience an aversive reaction, such as an expression of resolution. We reassessed our DP patients to see what lessons disgust, from others in response to the appearance of their we could learn from them. Between 1995 and 2006, we col- skin. We have reported previously that patients may be hyper- lected data on 12 patients with DP. The diagnosis was made vigilant for such signs of social stigma. Functional magnetic on the detailed history and clinical findings. Further reassess- resonance imaging (fMRI) of the brain is able to identify dis- ment was made by telephone contact with their family doctors tinct areas responsible for processing of different facial expres- and review consultations. Twelve patients, six male and six sions. For example, the insula and amygdala process disgust female, were identified with mean age of 46 years (24–74). and fear, respectively. We utilized fMRI to determine whether All patients were white. Four patients were married, two sep- the insula response to facial expressions of disgust differed arated and six were single. All were employed. The mean age between patients with psoriasis and healthy volunteers. Ten at presentation was 41 years (23–68) and the mean duration right-handed male patients (mean age: 35 ± 8Æ1; range: 23– of symptoms at first consultation was 55 months (3 months 44 years) with moderate or severe chronic plaque psoriasis, to 19 years). The mean duration of follow-up was 52 months. treated with topical and/or systemic medication (mean PASI: Four patients had consulted other dermatologists before 5Æ3±3Æ6; range: 1Æ1–12Æ2), and 10 age-matched male attending us. Folie a` deux occurred in two couples. Proof of the healthy volunteers (mean age: 29 ± 6Æ3; range: 21–41 years) infestation, such as specimens in a matchbox, was provided viewed grey-scale pictures of faces, from a validated set of by three patients. Seven patients had a history of previous prototype images, depicting either 100% disgust, 100% fear parasitic infestation diagnosed and treated by their family doc- or a neutral expression. Using a standard ‘box-car’ design we tors. Six patients had had previously attended psychiatrists alternated 37Æ5 second blocks of facial expressions depicting with depression. Although, the distressing itching and crawl- either disgust or fear with neutral faces. The fearful facial ing sensations were graphically described by all 12 patients, expressions were included as an aversive expression control. two were initially misdiagnosed by us. Three patients had sec- Brain images representing blood oxygen-level dependent ondary associated conditions (two with recreational drug use

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 56 Bristol Cup Posters and one Alzheimer’s disease). Seven patients had no skin 0 indicated normal skin and 10 the worst possible. The mean abnormalities. In five patients, excoriations, erythema and age of patients (n = 111, M = 48, F = 63) was 46 years (SD lichenification were observed. Eleven patients were treated =24Æ1); the mean age of family members (n = 111, M = 42, with antipsychotic drugs: 10 patients were treated with initial F = 69) was 52 years. The mean duration of patients’ skin low dose pimozide and only one cleared fully. The nonre- disease (n = 43) was 8Æ7 years (SD = 10Æ3, range 1 week to sponders had to discontinue because of dose-related extra- 55 years). Most family members were either parents (32%) or pyramidal side-effects. Four were treated with sulpiride with spouses/partners (56%). The mean DLQI score for patients one reported improvement. Eight patients were seen in the last was 8Æ2 (range 0–29, SD = 7Æ2) and their self-assessed disease 6 months with six still having active DP symptoms. Four were severity mean score was 5Æ3 (SD = 2Æ8). The mean FDLQI lost to follow-up. The treatment of DP is difficult as these score for family members was 7Æ6 (range 0–26, SD = 6Æ2) patients may be otherwise well but have a fixed unshakeable and their self-assessed patients’ disease severity score was 5Æ03 belief that they are infested. They almost always reject the (SD = 2Æ8). There was a high correlation between both the diagnosis and psychiatric referral. We were surprised by the DLQI and patient-rated disease severity (r =0Æ67, P <0Æ001) persistence of symptoms and poor response to therapy in our and the FDLQI and family member-rated patient’s disease patients. In our series, the side-effects of neuroleptics limited severity (r =0Æ59, P <0Æ001). We also found a high correla- treatment efficacy. Factors contributing to poor prognosis in tion between patients’ and family members’ subjective assess- our group included single or separated marital status, pro- ments of patients’ disease severity (r =0Æ71, P <0Æ001). The longed DP symptoms before referral and a past history of QoL of not only patients but also of their family members is depression (6 of 10). Patients did seem to appreciate the related to the self-assessed severity of the patients’ skin dis- chance to express the distressing nature of their symptoms ease. It is very important to consider the family perspective and an empathetic approach allowed a therapeutic relationship while making management decisions. to develop. However, our series underscores the need for liai- son psychiatric collaboration within the dermatology clinic.

P-77 The hidden cost of skin scarring P-76 B.C. Brown, S.P. McKenna,* K. Siddhi,* Quality of life of patients and their families is related D.A. McGrouther and A. Bayat to the self-assessed severity of patients’ skin dis- Manchester Medical School, The University of Manchester, Manchester, U.K.; ease *Galen Research, Manchester Science Park, Manchester, U.K.; Department of M. K. A. Basra and A.Y. Finlay Plastic Surgery, Wythenshawe Hospital, South Manchester University Hospital Cardiff University, Wales College of Medicine, Cardiff, U.K. Trust, Manchester, U.K. and Manchester Interdisciplinary Biocentre, Skin diseases are known to affect the quality of life (QoL) of The University of Manchester, Manchester, U.K. patients and their families (Basra MKA, Sue-Ho R, Finlay AY. Little is known about how scars affect patient’s lives. Specialist The Family Dermatology Life Quality Index: measuring the clinical impressions suggest their impact is related to their: (i) secondary impact of skin disease. Br J Dermatol 2007; 156: cause, (ii) physical symptoms and (iii) appearance. Facial scars 528–38). In children with atopic eczema the QoL of patients have been shown to cause high levels of anxiety and self and their families (Balkrishnan R, Housman TS, Carroll C et al. consciousness, but other aspects have been neglected. We Disease severity and associated family impact in childhood aimed to explore the effects of skin scars on patients’ quality atopic dermatitis. Arch Dis Child 2003; 88: 423–7) is related to of life (QoL) and identify potential implications for clinical the severity of disease. However, no study has investigated the practice. We performed qualitative in-depth interviews at a relationship of QoL of adult patients and their family caregiv- Plastic Surgery clinic, adopting a needs-based approach to ers with the self-assessed severity of skin diseases in general. QoL. All interviews were recorded on audiotape and tran- The aim was to examine the relationship of QoL of patients scribed verbatim, then underwent Interpretative Phenomenologi- and their immediate family members with self-assessed disease cal Analysis. Thirty-four patients with a scar (24 women; age severity in a cross-section of skin diseases. Patients attending 14–70, mean = 36, SD = 17Æ9 years) with a wide range of the dermatology out-patient clinic of a university hospital scar type, severity and onset were recruited. Transcripts of were asked to complete the Dermatology Life Quality Index interviews about their needs as affected by skin scars yielded (DLQI) while their accompanying family members/partners 567 statements. These were subsequently classified into 44 completed the Family Dermatology Life Quality Index themes covering five main areas: (i) physical comfort and (FDLQI), a novel validated generic dermatology-specific QoL functioning, (ii) acceptability to self and others, (iii) social measure for the families of skin disease patients. Both the functioning, (iv) confidence in the nature and management of DLQI and the FDLQI have a similar scoring system (score the condition and (v) emotional well-being. The majority of range 0–30). A global question was included at the end of respondents were unhappy with the appearance of their scar both questionnaires asking the respondents their assessment of because of the perceived stigma and psychological associa- the patients’ disease severity on a 0–10 visual analogue scale; tions, and thus they adopted different coping behaviours to

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 57 hide or compensate for the scar. Often this made them unso- with eczema and psoriasis accounts for one third of outpatient ciable and interfered with their communication skills, personal clinic visits (excluding photo-therapy and dressings). Referrals relationships, work life and leisure activities. Concerns about from other hospital specialties have doubled to 10% of the the diagnosis and persistent nature of scars were common, total since 1980. These results confirm the demand from both whilst lack of empathetic management by general physicians Primary and Secondary Care for a specialist dermatology ser- and the frustrations of current treatment compounded distress. vice. There are five main areas of effect on the needs of patients with skin scarring that should be addressed in their manage- ment; these are greater and more complex than is generally thought. Education of clinicians will raise awareness of the influence of scars on QoL and improve their management. P-79 Public education may also lessen the stigma attached to scars ‘Choose and Book’ and dermatology referrals: and help to reduce distress. Support services should be avail- experience so far able when patients’ social functioning is impeded, which J.K. Schofield, L. Mascia, C. Lewis and A. Griffiths effects their emotional well-being. Our results indicate the West Hertfordshire NHS Hospitals Trust, St Albans and Hemel Hempstead, need for a carefully designed measure of QoL with high psy- U.K. chometric properties, for use in clinical settings and trials. We Giving patients more choice about how, when and where they have developed a scale from the findings of this study, which receive treatment is a cornerstone of the U.K. Government’s has been field-tested and is currently under validation. This health strategy. To facilitate this the so-called ‘Choose and will be previewed at presentation. Book’ program is a national service that combines electronic booking and a choice of place, date and time for first outpa- tient appointment. The referral letter is then sent electronically via the general practitioner (GP) to the chosen provider who P-78 accepts or rejects the referral. The aim of this study was to Dermatology workload: twenty-five years on evaluate the introduction of the ‘Choose and Book’ process on E.C. Benton, O.A. Kerr, A. Fisher, S.J. Fraser, dermatology referrals in our locality. Our electronic booking S.K.A. McCormack and M.J. Tidman project commenced in October 2004. All dermatology services The Edinburgh Royal Infirmary, Edinburgh, U.K. were listed on the directory of services (DOS), except for 2- In this, the fifth audit of dermatology services since 1980, we week skin cancer referrals, from July 2005. By January 2006, aimed to quantify the out patient workload in hospital-based 12 GP surgeries in our locality had software installed and and private practice, to assess the reasons for referral to Secon- training completed in order to be able to use the facility. For dary Care and to examine the changes over 25 years in the the purpose of this study, the total number and type of refer- diagnostic spectrum of the conditions most commonly rals (electronic or paper), whether or not the electronic refer- referred. During November 2005, all outpatient consultations rals were accepted or rejected and any reason for the latter, in a region serving 1Æ2 million people were recorded. Demo- were collected for the 11 month period January to November graphic data, source of and reason for referral, waiting times, 2006. Overall dermatology referral rates in the 11 month per- diagnosis, investigations, treatment and disposal were recorded iod rose steadily from between 138 and 226 per month in the in standard format. Diagnoses were coded manually, after first 7 months of the study period to between 318 and 360 histological examination if appropriate: data were then per month in the final 4 months. Uptake of Choose and Book scanned into an Access database. The total number of consult- referrals in the first six months was slow but by the end of ations had increased by 67% since 1980. In November 2005, the 11 month period 43% of all dermatology referrals (exclud- 2118 new and 2796 review consultations were available for ing 2-week-wait referrals) were being received electronically. analysis (new : review 1 : 1Æ3). Women predominated (F : M The increase in total dermatology referrals coincided with the 1Æ3 : 1); age 0–105 years, (mean 49 years for new patients increase in the number of Choose and Book referrals. Of a and 53 years for review). Ninety per cent of new referrals total of 427 referrals received electronically, 50 (12%) were came from Primary Care, 10% from Secondary Care. The rea- rejected by the clinicians on receipt of the referral letter. The sons for referral were for a diagnostic opinion in 57% of cases commonest reason for this was patients being booked into a and management advice in 38%. Trends in the most com- routine appointment slot when an urgent appointment was in- monly referred conditions seen during the 5 audits are tabula- dicated based on the subsequent clinical information received ted. Of return patients, skin cancer accounted for 20%, in the referral letter (30 of 50). Most of the referrals rejected eczema 16%, psoriasis 13%, acne 9% and viral warts 4%; the were subsequently rebooked into appropriate slots either via corresponding figures in 1980 were skin cancer 4%, eczema electronic or paper referral (38 of 50). This study provides 19%, psoriasis 10%, acne 4%, and viral warts 33%. In conclu- some preliminary evidence that the Choose and Book process sion, attendances for both benign and malignant skin tumours has increased dermatology referrals in our locality. For the have increased six-fold since 1980. Management of patients most part patients are booked appropriately although

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 58 Bristol Cup Posters modification of the DOS is necessary to ensure that patients P-81 are booked with the correct priority. Dermatology follow-up caseload: national recom- mendations, local problems P-80 J.K. Schofield, T. Adlard and S. Gunn An audit of remote supervision of a general practi- West Hertfordshire NHS Hospitals Trust, St Albans and Hemel Hempstead, tioner with special interest via teledermatology U.K. C. Thind, I. Brooker* and A.D. Ormerod The system of payment by results introduced in April 2006 Aberdeen Royal Infirmary, Aberdeen, U.K.; *General Practitioner, McDuff, requires that all activity performed by secondary care organi- U.K.; and University of Aberdeen, Aberdeen, U.K. zations should be paid for according to the agreed national Patients referred to a community hospital 50 miles from the tariff. The White Paper published in January 2006 (Our Health, dermatology centre were selected jointly by a dermatologist Our Care, Our Say: a New Direction for Community Services. Department and GP with Diploma in Dermatology training as a general of Health, 2006) also gave recommendations with respect to practitioner with special interest (GPwSI) (IB). Consenting reducing new to follow-up (FU) ratios to the low decile of patients were seen by IB and a diagnosis and treatment plan 1:1Æ53. The aim of this study was to review in detail our FU formulated. All patients were photographed and referred elec- caseload in the context of the commissioning intention of our tronically by store and forward teledermatology including local primary care trust of a ratio of 1 : 1Æ2 compared with dermoscopic images, monthly to the consultant for opinion, our current ratio of 1 : 1Æ6. In particular, we wanted to review and feedback. The project complemented traditional explore whether this ratio can be achieved while meeting monthly joint clinics and assessed the value of teledermatology national clinical guidance and recommendations. We reviewed in remote supervision and education for GPwSIs. Over a 2-year the total number of FU appointments required by patients period 230 teledermatology patients (102 males and 128 overall and then with respect to different diagnostic groups. females) in the age range 1–106 years (median 54) and a new All FU data for a 12-month period was collected from our to review ratio of 4 : 1 were audited. There was good agree- Infoflex clinical information system. This was then analysed in ment on diagnosis in 60%. Auditing diagnostic agreement was Microsoft Excel. No FU was arranged for 36% of patients and difficult and complicated by poor images, awaited biopsies, 35% were reviewed only once. The top 10 diagnoses of those multiple pathologies and diagnostic uncertainty for the GP or needing more than one FU appointment are shown below. the specialist. Of the patients 70% did not require follow-up We believe that we are following national recommendations by the dermatologist. Educational feedback to GPwSI occurred for review of patients with MM, SCC, LS, acne (isotretinoin) in 66% using opportunities to add training wherever possible. and patients with psoriasis and eczema on second-line treat- Satisfaction surveys of 50 consecutive patients with 31 patient’s ments. Earlier discharge of patients with BCC and AK may responses showed 28 were very or completely satisfied. There provide some capacity to reduce FU activity. This study shows was equipoise in patients’ preference to see a dermatologist or that achieving a new to FU ratio that is less than that recom- a GPwSI. A detailed analysis will be presented. On the down- mended in the White Paper will be difficult to achieve where side, two melanomas were detected but diagnostic agreement referrals are appropriate and national guidance is followed. was poor for this diagnosis. Setup costs were £12,000. We had significant down-time because of system breakdowns and poor Mean no. Total no. technical support. The software was functional but terminated No. of of FU in FU slots in when Millennium went into liquidation. Teledermatology was Diagnosis patients 12 months 12 months time consuming, requiring an average of 20–30 minutes per Basal cell carcinoma (BCC) 328 1Æ52 499 patient. Additional time was taken for review of images by the Eczema/dermatitis 325 1Æ94 630 consultant (2 hours per month) but less time was spent travel- Psoriasis 253 2Æ17 549 ling. An important feature of this model is that it applied only Squamous cell carcinoma (SCC) 207 1Æ88 207 to preselected cases, which the investigators felt were within Melanocytic naevi 206 1Æ37 282 IB’s competence. Cases were simple or conducive to telederma- Acne 199 1Æ92 382 tology assessment. In a randomized trial of teledermatology, Actinic keratosis (AK) 199 1Æ59 316 Malignant melanoma (MM) 95 1Æ91 181 (Bowns IR, Collins K, Walters SJ, McDonagh AJ. Telemedicine Lichen sclerosis (LS) 70 1Æ84 129 in dermatology: a randomised controlled trial. Health Technol Leg ulcers 64 2Æ25 144 Assess 2006; 10: 1–58) agreement on diagnosis and manage- ment was lower at 55% which was inferior compared with face to face consultation but used GPs without additional dermatology training. However, we had a single trained GP P-82 working with the same consultant on preselected cases in a Audit of cutaneous surgery by specialty remote setting. Of these patients, 70% did not have to travel, S. Bashir and J.S. Ross or conversely, consultant travel was reduced. In selected University Hospital Lewisham, London, U.K. patients, teledermatology was a useful tool for supervising Cutaneous surgery is presently performed by a variety of pro- GPwSI clinics in a remote area. viders other than dermatologists, in both primary and hospital

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 59 settings. Local guidelines may limit benign and cosmetic pro- vative model of service delivery which offers patients a self- cedures, while national guidelines direct skin cancer surgery. referral, ‘pay-as-you-go’ service. This consultant-led scheme The aim of this audit is to quantify the extent of skin surgery runs in parallel to the secondary care dermatology services and in our locality, the types of lesions being removed, who is offers all procedures previously excluded by the local PCT for performing procedures and their diagnostic accuracy. Data cosmetic reasons: mole scanning, Botox for axillary hyperhid- were gathered retrospectively by analysing histology reports rosis and routine dermatology consultations for patients who for a 5-week period from 1 March 2006. Data obtained inclu- wish to be seen locally and are not currently able to be seen ded: specialty of operator, clinical diagnosis, type of proced- within secondary care services because of reduced capacity. ure and histological diagnosis. The results showed that 222 Purely cosmetic advice and treatments are also available within skin surgical procedures were performed in the study period. this service and include photorejuvenation consultations and One hundred and twenty-eight (40%) procedures were per- total skin-care programmes with/without skin imaging. formed by dermatologists, 61 (27%) procedures were per- Patients can access dermatologist-recommended skin products formed by surgeons and 33 (15%) were performed by (evidence-based products available either by prescription or general practitioners. Fifty-one per cent of the dermatology over the counter) from our pharmacy. Rejuvenating procedures procedures were on benign skin lesions, compared with 91% on offer include: antiwrinkle injectables, e.g. dermal fillers, dy- of general practice lesions and 82% of the surgical depart- namic wrinkle relaxation injections and also intense pulsed ment’s lesions. Diagnostic accuracy was 89% for dermatolo- light (IPL) treatments for photorejuvenation/fine lines. IPL is gists, 76% for ENT surgeons (17 lesions), 76% for general also available for hair removal and removal of vascular and surgeons (33 lesions), 100% for paediatric surgeons (eight pigmentation problems. This service has received interest at a lesions) and 100% for other surgical specialties (three lesions). local, national and international level. This novel model of ser- The diagnostic accuracy was lowest for general practitioners at vice delivery has now been running as a pilot for 10 months. 61%. Our findings show that a large number of biopsies are We outline the stages involved in setting up this service, performed by nondermatologists. The diagnostic accuracy of review the cases and caseload that have been seen, illustrate the dermatologists is higher than all other specialties, although, economics involved in the management, development and run- when surgical specialties are subdivided into those performing ning of this service. We also present the extremely high patient very few specific procedures (e.g. paediatric surgery and satisfaction that has accompanied this innovative process. It is removal of dermoid cysts) some subspecialties do better. clear that patients welcome the opportunity to self-refer, However, given the breadth of diagnosis of skin lesions, we appreciate attending an NHS hospital environment for cosmetic believe that dermatologists are best placed to diagnose and treatment and above all fully support the fact that income gen- choose an appropriate method of treatment, if required. As erated is placed back into the NHS (not into the private sector) nondermatologists are removing a high percentage of benign – with a proportion of the profits being earmarked for the Brit- lesions, there is a potential cost-saving opportunity by having ish Skin Foundation. Models of dermatology care are currently these lesions screened by dermatologists. Further, the NICE being examined across the U.K. We present this as a successful guidelines on improving skin cancer outcomes require that all innovative option to consider. doctors treating skin cancer be part of a multidisciplinary team, and that high-risk lesions be treated in a hospital set- ting. Doctors who do not fulfil these criteria are working out- P-84 with the NICE recommendations. Dermatologists are well The pros and cons of copying patients into their GP placed to treat skin cancer as we have a high diagnostic accur- letters: 7 years’ experience acy and form an integral part of the multidisciplinary team. K. Baxter and A.M. Layton* Foundation Skin and *Department of Dermatology, Harrogate and District NHS Foundation Trust, Harrogate, North Yorkshire, U.K. P-83 The NHS Plan (paragraph 10.3) states that ‘letters between cli- Foundation Skin – a novel innovative service in nicians about individual patient care will be copied to the support of secondary care dermatology patient as a right’. The aim is to improve communication K. Baxter, A. Boston and A.M. Layton* between doctors and patients and involve patients in their Foundation Skin and *Department of Dermatology, Harrogate and District healthcare, encouraging a ‘spirit of openness’ that underpins NHS Foundation Trust, Harrogate, North Yorkshire, U.K. the Data Protection and Freedom of Information Acts. Sending The current political climate which includes the Government’s patients copies of their general practitioner (GP) letters is launch of ‘care closer to home’, the introduction of payment regarded as good/best practice and ways to implement this by results and the commitment of the Government to intro- across the healthcare system are currently underway. We detail duce private influences into the healthcare arena are all going our impressions from 7 years’ experience of this practice and to have a significant impact on secondary care services. In con- illustrate both points in favour of and against this approach. junction with the financial problems of the primary care trusts Points in favour: patients are pleased to be involved in their (PCTs) there is a large incentive to reduce the number and/or healthcare and to be part of an open and honest approach. cost of referrals reaching secondary care. We describe an inno- Clear information helps them understand the condition/

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 60 Bristol Cup Posters treatment better. Patients can refer back to the letter after an format of the questionnaire was agreed and sent to consecutive appointment – it is well recognized that information is poorly patients attending clinics between January and February 2005. retained after a consultation. Patients reflect on suggested self- Also enclosed was a copy of the patient’s letter to the GP (dic- help and lifestyle changes and this can be an impetus to seek tated following their appointment), an explanatory cover letter help to change. Patients use the information provided to find and a freepost return envelope. The questionnaires were anony- out more about their condition which in turn generates well- mous and patients were advised that it was not obligatory to informed and motivated patients. For all concerned this ap- complete the questionnaire. A total of 112 new patient ques- proach encourages clear communication of health issues and tionnaires and 57 follow-up patient questionnaires were sent; accountability. Other healthcare teams (e.g. community nur- 40% and 35% were returned, respectively. Ninety-five per cent ses, pharmacists) and carers benefit. The included ‘layman’ (i) were pleased to receive a copy of their GP letter, (ii) valued terminology can be educational. This approach appears to seeing the information sent to their GP, (iii) wished to con- minimize unnecessary repeat nursing and secretarial enquiries tinue to receive copies of their GP letters and (iv) planned to as all aspects of the patient’s clinical and clerical journey is keep their letter. Eighty-eight per cent found the letter helped documented – from the medical management plan to appoint- them understand the treatment(s) for their condition. Three ments. Documentation of risks associated with planned pro- felt that the treatment had already been fully explained verbally cedures/treatments aids the informed consent process and and one patient was being investigated so treatment discussion letters may prove useful should litigation arise. Specialities was delayed. Eighty-five per cent felt the letter helped them such as mental health and paediatrics have some of the most understand their diagnosis better. Of the ‘no’ answers, three complex of cases yet are at the forefront of patient-letter wri- patients felt that the letter did not help them understand the ting so complex/sensitive problems need not be a barrier. diagnosis further as it had been fully explained at the appoint- Points against and challenges: the most significant problems ment. Seventy-eight per cent felt the letter helped them relate to time and cost. Letters directed to patients with pertin- remember the treatment(s) for their condition. Of the four ent information take longer to dictate, type and sign. Cost im- ‘no’ responses, all wrote that they had no problems remember- plications include extra resources for staff and postage. Patient ing what had been said at the consultation. Overall, 73% had confidentiality and data protection issues need careful consid- found the letter very helpful, 23% found it a little helpful; 63% eration and format consideration is required especially for spe- found the letter very easy to understand, 29% found it reason- cial needs, e.g. the visually impaired. Copying of patient ably easy to understand. Only 6% had ever received a similar letters is a controversial area with valid arguments both for letter previously. There is increasing pressure on clinicians to and against (Young-Min SA, Hamilton J, Heycock C, Kelly C. provide patients with copies of GP letters. Accurate, thorough Copying clinic letters to patients will place additional demands documentation has always been a vital part of practitioners’ on both secondary and primary care: a hospital outpatient working lives. Dermatology lends itself well to this process and questionnaire. Rheumatology (Oxford) 2004; 43: 1306–7). Some adopting this approach clearly helps in clarifying treatment specialities lend themselves better to this process and, with the regimes for patients. The patients appreciate the honesty and appropriate infrastructure, dermatology is one of these. respect, and understand their proposed investigation and man- agement. Community practitioners have supported the clarity of these letters and have found the content educational. From a P-85 personal perspective this approach forces clear thought proces- Patients’ views on receiving copies of their GP ses and commitment to a management plan. A repeat of this letters local service evaluation has recently been conducted using a K. Baxter, M. O’Connell* and A.M. Layton* different population base. Within this latter assessment, time Foundation Skin and *Department of Dermatology, Harrogate and District for service development and costs have also been analysed, NHS Foundation Trust, Harrogate, North Yorkshire, U.K. along with staff and patient satisfaction. It is clear that there are Sending patients copies of their general practitioner (GP) letters funding issues that need to be taken into account if this ap- post-consultation was deemed a government initiative/objec- proach is to be supported across the speciality. tive within the new NHS Plan (paragraph 10.3). This states that ‘letters between clinicians about individual patient care will be copied to the patient as a right’. Some dermatologists routinely P-86 send letters to their patients but others do not adopt this ap- The impact of NICE guidance upon the surgical proach. Different geography, service commitments and funding management of skin cancer within primary care inevitably influence this decision. Personal experience over the K. Gordon, M. Petkar,* M.G. Cook* and A. Marsden last 7 years suggested that sending patients copies of their GP St George’s Hospital, London, U.K. and *Royal Surrey County Hospital, letters resulted in very positive feedback, aided compliance and Guildford, U.K. reduced patient enquiries following their dermatology appoint- This study aimed to evaluate the impact of recent NICE guid- ment. The aim of this local service evaluation was to assess ance upon surgical management of suspected skin tumours by patient opinion formally with a postal questionnaire. Approval general practitioners (GPs). Skin specimens submitted by GPs was obtained from the Clinical Effectiveness Department. The for histopathology to a district general hospital (DGH) were

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 61 analysed. Data were collected from September to October in However, their educational needs must be balanced with 2006 and 2005. In addition to the volume and nature of spec- respect for patients’ wishes and the provision of a comfort- imens received, we compared the GPs’ clinical diagnoses to able, patient-centred environment. Our survey was conducted the corresponding histopathological diagnosis. A total of 147 in a general adult dermatology clinic of a teaching hospital to specimens were received during the 2-month period in 2005. assess patients’ attitudes towards medical students’ presence Of these, 125 (85%) were clinically and histologically benign. during their consultation. Of 65 patients invited to participate, Thirteen cases (9%) were thought to be malignant and this 61 completed an anonymous self-administered questionnaire was confirmed on histology. Four lesions (3%) were suspected after their clinic appointment. Forty-three of these (70%) skin cancers that proved benign. Five cases (3%) were pre- acknowledged having been asked permission for the student sumed to be benign lesions by GPs, but routine histology to be present, whereas 18 patients (30%) were not aware of demonstrated malignancy, including two malignant mela- specifically giving their permission. Most patients (82%) were nomas. The number of specimens received in September– unaware of the presence of students when they entered the October 2006 increased by 22% to 179 cases. One hundred clinic room despite a sign being present on the door. Never- and forty-nine cases (83%) were clinically and histologically theless, most (72%) felt that they had a choice regarding the benign. Twelve cases (7%) were thought to be malignant and subsequent presence of medical students. The presence of stu- this was confirmed on histology. Five lesions (3%) were sus- dents did not seem to have a major impact on how comfort- pected malignancies that proved benign on histology. The able patients felt in disclosing personal information (83% number of presumed benign lesions that proved malignant in- agree or strongly agree). Sixty-four per cent did not feel that creased to 13 cases (7%), including three malignant mela- they required time alone with the doctor and a small minority nomas. Of note, none of the five malignant melanomas were (13%) recorded that they did not enjoy their interaction with suspected clinically in either group. Our data demonstrate the medical students. Interestingly, 40% of patients felt they increasing trend for GPs to perform minor surgery in the gained greater understanding of their condition because med- community. The number of misdiagnosed malignant lesions ical students were being taught in clinic. Our survey has within the community markedly increased within 12 months. shown that despite being a teaching hospital, it is still import- Our study also revealed that GPs have a high rate of incom- ant that patients are clearly informed of the presence of med- plete excisions, with 50% of all malignant lesions being ical students in clinic and that they have the option of being incompletely excised between September and October 2005. seen without students. Reassuringly, most patients enjoyed These findings highlight the necessity of sending all skin sam- their interaction with medical students in the outpatient clin- ples for histological examination, unlike the current practice ics. of many GPs who do not routinely obtain histology of lesions they believe are benign. GPs within the catchment of this DGH have disregarded NICE guidance on the management of P-88 skin cancer, as evidenced by continuing to treat or investigate Vulval lichen sclerosus: the case for a specialist a number of suspected malignancies, albeit nonmelanoma skin vulval clinic cancer, surgically in the community. NICE guidelines clearly M. Mehmi, K. Shalders and S. Velangi state that doctors who knowingly treat skin cancer in the com- Birmingham Skin Centre, Birmingham, U.K. munity should be members of a multidisciplinary team Lichen sclerosus (LS) in female patients is frequently man- (MDT), attending a minimum of four meetings per year. aged in secondary care. A process and standards based None of the GPs included in this study were members of the retrospective survey of patient notes with a histological local skin MDT. In light of our recent findings, further work diagnosis of lichen sclerosus during 1996–2006 was con- is being undertaken to evaluate the surgical management of ducted in our department. The survey aimed to assess varia- skin cancer by GPs over the last 3 years. We believe there is tions in practice against the British Association of need to highlight the discrepancies between GP practices and Dermatologists guidelines prior to the introduction of a spe- NICE guidance especially in light of the recent trend to move cialist vulval clinic (Neill SM, Tatnall FM, Cox NH. Guide- dermatology services into the community. lines for the management of lichen sclerosus. Br J Dermatol 2002; 147: 640–9). Thirty-six patients aged between 11 and 91 years (mean age 52 years) were identified. Twenty- P-87 four patients had genital LS, eight had extragenital disease, Patients’ attitudes towards medical students’ and four female patients had both. Fifteen patients (42%) presence in dermatology outpatient clinics were seen by a dermatologist, 20 (56%) by a gynaecologist C. Bayliss, I. Chan* and S.H. Wakelin* and five (14%) patients were seen by both (one patient Imperial College Medical School, London, U.K. and *Department of Derma- was seen by a colorectal surgeon). The commonest present- tology, St Mary’s Hospital, London, U.K. ing symptoms were itching (53%) and soreness (31%); Medical students require contact with dermatological patients nine patients (25%) were asymptomatic. Psychosexual dys- as part of their undergraduate training. This has traditionally function was only discussed with one patient. Twenty been acquired by attending dermatology outpatient clinics. patients (56%) were initially prescribed a very potent

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 62 Bristol Cup Posters corticosteroid, six (17%) a potent corticosteroid and five respectively, in 2000. Sixty-six per cent (n = 37) provided (14%) asymptomatic patients were not given anything top- annual skin cancer surveillance compared with 21% in 2000 ically. The frequency and time period of corticosteroid use (P <0Æ05%, v2 test); 81% (n = 30) of screens were conduc- was documented in 60% of patients. Only eight patients ted by nondermatologists, nine (30%) of whom had formal (22%) were given advice about soap substitutes. One patient training for the role. Thirty-nine per cent (n = 22) offered full required surgery for apareunia secondary to scarring. Of 31 skin examination compared with 20% in 2000. A total of patients (86%) who were offered follow-up, 25 patients 69% (n = 31) of Australian centres responded. Skin cancer were seen within 3 months in accordance with guidelines. education was provided by 94% pre- and 87% post-transplant- Twenty-one patients (58%) were reviewed at least twice, ation; 97% (n = 30) provided skin cancer surveillance; 40% but did not attend (DNA) rates were high (22%). Malig- (n = 12) of screens were conducted by nondermatologists, of nancy risk was only mentioned in four (11%) cases. Risk which two (27%) had received formal training; 61% (n = 19) of loss of architecture and function with inadequate cortico- offered full skin examination. Despite global recognition that steroid use were not documented. Patient support groups renal transplant recipients should be educated about and were not mentioned at all. Patient information leaflets were receive surveillance for skin cancer and a substantial improve- only given in four cases. None of the nine discharged ment in U.K. practice between 2000 and 2006, one-third of patients was advised to see their GP for yearly review. U.K. centres still do not offer surveillance. Also there contin- Malignancy secondary to LS did not occur in this small ues to be a reliance on nonspecialists to provide screening. In sample. Most patients received appropriate first-line treat- Australia, where over 70% of RTRs will be affected by non- ment and follow-up. Documentation of discussion of psy- melanoma skin cancer after 10 years, virtually all centres offer chosexual symptoms and availability of patient information surveillance with more frequent specialist involvement. Vari- and support groups was poor. Lack of emphasis on poten- able frequency of skin cancer between and within populations tial complications, especially malignancy, may have contri- suggests that annual surveillance may not be required for all buted to the high DNA rate. The need for a GP annual RTRs. Predictive models of skin cancer risk may allow the review should be explained to patients before discharge. rationalization of screening in both temperate and subtropical Introduction of a tertiary vulval clinic with appropriate nur- climates and optimize the use of limited dermatological sing support should improve standards of documentation resources. and patient advice. We plan a prospective audit 12 months after introduction of the specialist clinic. P-91 Skin care in renal transplant patients S. Gossain, S. Keohane, S. Ozdemir* and T. Leach* Dermatology, St Mary’s Hospital, Portsmouth, U.K. and *Renal Unit, Queen P-89 Alexandra Hospital, Portsmouth, U.K. Abstract withdrawn. Renal transplant recipients have a greater than 40% cumula- tive risk of nonmelanoma skin cancers by 20 years after transplant and the incidence ratio of basal cell carcinomas P-90 (BCC) to squamous cell carcinomas (SCC) is reversed in this Skin cancer surveillance in renal transplant group. The increased risk for melanomas is 3Æ8- to 8-fold. recipients: re-evaluation of U.K. practice and Cumulative sun exposure to ultraviolet radiation is the comparison with Australian experience major risk factor but immunosuppression and human papil- S. Garg, R.P. Carroll,* R.G. Walker,* H.M. Ramsay loma virus are likely associated cofactors in transplant skin and P.N. Harden carcinogenesis. Insufficient dermatological surveillance and Department of Dermatology, Royal Hallamshire Hospital, Sheffield, U.K.; patient knowledge of these increased risks are likely also to *Department of Nephrology, Royal Melbourne Hospital, Melbourne, Australia play a role. The aim of our study was to assess the aware- and Department of Renal Medicine, Churchill Hospital, Oxford, U.K. ness of patients to their increased risk of skin cancers after The objective of this study was to determine skin cancer sur- renal transplantation. Our patient population was unique in veillance practices for U.K. renal transplant recipients (RTRs) that it comprised Navy and ex-Navy recruits, who had and compare with previous U.K. and current Australian data. excessive sun exposure, as well as civilians. All patients who A postal survey in 2000 established that only 21% of U.K. had a renal transplant between May 2004 and May 2005 RTRs received surveillance for skin cancer (Harden PN, Reece were recruited. Failed transplants and those who died (11 SM, Fryer AA et al. Skin cancer surveillance in renal transplant patients) were excluded. Questionnaires, based on three recipients: questionnaire survey of current UK practice. BMJ main themes, were sent to the remaining 53 patients. Three 2001; 323: 600–1). In 2006, a modified questionnaire was questions were based on skin care information, nine on sent to 84 U.K. and 45 Australian centres that follow up RTRs. skin cancer awareness and two on follow-up. Forty A total of 67% (n = 56) of U.K. centres responded in 2006. responses were returned. Eighty per cent of patients had Of these, 95% educated RTRs about the risk of skin cancer received information from the transplant unit on skin care pre- and post-transplantation compared with 63% and 88%, and photoprotection; 29% was verbal only, 21% was in the

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 63 form of a leaflet and 50% was both verbal and a leaflet. consensus statement. Br J Dermatol 2004; 150 (Suppl. 67): 11– Seventeen per cent of patients felt the information was 23). The Cr51 EDTA clearance was performed 22 times over insufficient. Sixty per cent of patients knew about the the 5-year period from January 2000 to December 2005, on 17 importance of photoprotection and the increased risk of patients receiving ciclosporin for over a year for dermatological skin cancer; 23% knew they had an increased risk of skin conditions. The majority of the cohort had psoriasis (65%). cancer due to ultraviolet radiation and immunosuppression. The remainder had chronic ordinary urticaria (12%), nodular Three per cent of patients mentioned skin colour as being prurigo (12%), atopic eczema (6%) and Behc¸et’s disease (6%). important in view of their risk of skin cancer. Eighty-seven One patient had pre-existing hypertension, diabetes mellitus per cent of patients knew they needed to use a sunblock of and bipolar depression treated with lithium. None of the other factor 25 or more and 67% knew that the sunblock needed patients had risk factors for, or a history of, renal disease. Ten to be reapplied 2-hourly and after swimming; 97% thought patients were men and seven women. The mean age of the that photoprotection was not required on a cloudy day and group was 46 years. Five out of 22 measurements of GFR by 87% knew that photoprotection was required when in Cr51 EDTA led to a recommendation to reduce the dose of ci- water or on snow because of ultraviolet reflection; 100% closporin, despite the percentage change in the serum creati- knew they should avoid the sun between noon and 15Æ00. nine level being < 30% compared with baseline. In two out of Only 3% reported they would contact a dermatologist if 22 measurements of GFR by Cr51 EDTA the result led to a they noticed a changing or bleeding mole; 70% stated they recommendation to leave the dose of ciclosporin unaltered would contact their GP and 3% would do nothing. Sixty despite the serum creatinine increasing by > 30% compared per cent had been seen by a dermatologist since their renal with baseline. Reliance on percentage change in serum creati- transplantation and of those 72% were seen within 6 nine compared with baseline in assessing long-term ciclosporin months; 3% did not know if they had seen a dermatologist. nephrotoxicity may lead to inappropriate adjustments of ciclo- In conclusion, skin cancer awareness amongst renal trans- sporin therapy. Cr51 EDTA clearance should be considered to plant recipients could be improved, particularly their know- monitor for nephrotoxicity in those on long-term ciclosporin. ledge on skin surveillance and changing moles and the role of the dermatologist. This may be improved if provided in a specialist dermatology setting. P-93 Skin cancer awareness in renal transplant patients A.-M. Skellett, M. Andrews and J. Garioch Norfolk and Norwich University Hospital NHS Trust, Norwich, U.K. P-92 Patients who have a transplanted organ are 200 times more Influence of the measurement of renal function by likely to develop skin cancer compared with age-matched con- chromium-labelled ethylenediaminetetraacetic acid clearance compared with serum creatinine on long- trols (Moloney FJ, Comber H, O’Lorcain P et al. A population- term ciclosporin therapy based study of skin cancer incidence and prevalence in renal N. Rajan and A.J. Carmichael transplant recipients. Br J Dermatol 2006; 154: 498–504). Sun Department of Dermatology, The James Cook University Hospital, Middles- exposure is an important contributory risk factor. Patients with brough, U.K. transplanted organs need to be educated on the risks of sun Ciclosporin is an effective agent in the treatment of psoriasis exposure and to be taught self-monitoring so that the onset of and other inflammatory dermatoses. Its most significant adverse skin cancer can be minimized or at least be detected at an effect is nephrotoxicity, which is dependent on dose and dur- early stage. In order to assess awareness of the risks of sun ation of therapy. Accurate monitoring of renal function is cru- exposure and the development of skin cancer in renal trans- cial in patients treated with ciclosporin. The accepted gold plant patients in our hospital, we performed a postal survey of standard for the measurement of glomerular filtration rate all those under the care of the renal team. Questionnaires were (GFR) is chromium 5-labelled ethylenediaminetetraacetic acid sent to 178 patients. The questionnaire was based on the in- (Cr51 EDTA) clearance (Chantler C, Garnett ES, Parsons V, Veall formation leaflets supplied by the British Association of Der- N. Glomerular filtration rate measurement in man by the single matologists and the regional transplant centre on skin cancer injection methods using 51Cr-EDTA. Clin Sci 1969; 37: 169– in the immunosuppressed. The questionnaire was returned by 80). We report the results of a 5-year audit of the influence of 124 patients. The results were as follows: 81% of patients had the measurement of renal function by Cr51 EDTA clearance on been given advice on sun protection, usually by their renal long-term ciclosporin therapy, compared with percentage physician or nurse; 40% stated that they did not wear a hat change in serum creatinine. A GFR of < 60 ml min–1 (U.K. and 34% said they did not cover up arms and legs; 70% chronic kidney disease stage 3), measured by Cr51 EDTA, was patients said they applied sun cream to all exposed areas but used as the threshold to reduce the dose of ciclosporin. Current 40% did not know about the star rating of sun creams. Writ- dermatology guidelines recommend a reduction in the dose of ten information on the risks of skin cancer was only given to ciclosporin if serum creatinine increases by 30% compared 44% of patients. Of those who had received written informa- with baseline measurement (Griffiths CE, Dubertret L, Ellis CN tion, only 57% were happy with the information supplied. et al. Ciclosporin in psoriasis clinical practice: an international Patients in our survey had a reasonable overall knowledge of

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 64 Bristol Cup Posters the risks of skin cancer following a kidney transplant. Many delivery of optimal care by dermatologists is expensive and said that they would like to be provided with more written has implications for resources in an already overstretched information. In response to this, we are developing an infor- health service. Perhaps newer immunosuppressive agents such mation leaflet relevant for our local transplant population. We as sirolimus with antiproliferative effects will reduce the can- are planning to train a renal nurse to educate patients and to cer burden in the future, but for now regular dermatology recognize premalignant and malignant skin lesions. By doing review is essential to deliver high-quality patient care. this, we hope to minimize our patients’ risks of developing a skin malignancy, or at least ensure that they are detected early. P-95 in renal transplant recipients A. Lally and F.T. Wojnarowska P-94 Department of Dermatology, Oxford Radcliffe Hospitals, Oxford, U.K. The cost of delivering a skin cancer service to renal Seborrhoeic dermatitis is an inflammatory dermatosis affecting transplant recipients 2–5% of the general population. It is more common in indi- A. Lally and F.T. Wojnarowska viduals infected with human immunodeficiency virus (HIV). Department of Dermatology, Oxford Radcliffe Hospitals, Oxford, U.K. However, its prevalence in other immunosuppressed groups is Improvements in transplant immunology and surgical tech- unknown. The aim of this study was to look at the prevalence niques have resulted in enhanced survival for renal transplant and pattern of seborrhoeic dermatitis in renal transplant recip- recipients (RTRs). RTRs, particularly those of Northern Euro- ients (RTRs). RTRs attending a tertiary renal transplant unit pean descent, have up to a 100-fold increased risk of nonmel- were invited to participate. Information on demographics, anoma skin cancer. These tumours are often multiple and transplant history and dermatological history was obtained by relatively aggressive. The recently published NICE guidelines completion of a questionnaire. Detailed skin examination was on cancer services estimate that the cost of employing staff for carried out. When seborrhoeic dermatitis was identified clinic- transplant patient skin clinics is likely to be £4370 to £17 480 ally, exacerbating and relieving factors, and distribution and per transplant unit. This does not take into consideration the severity were recorded. Between May 2005 and August 2006, cost of surgery or other treatments required by this patient 396 RTRs were invited to participate and informed consent group. SCOR (skin care in organ recipients) U.K. recommends was obtained from 308. Twenty-nine (9%) were found to that all RTRs are seen annually from 1-year post-transplant have seborrhoeic dermatitis clinically. Seborrhoeic dermatitis and more frequently once a skin cancer has been diagnosed. was more common in older RTRs, with a mean age of 56 ± The aim of this study was to investigate the cost of delivering 15 years vs. 51 ± 13 years in the control group, and also in an optimum dermatology service. The burden of premalignant those who had been transplanted for longer (mean duration and malignant cutaneous disease amongst RTRs attending a of transplant 14 ± 7 years compared with 10 ± 8 years). tertiary referral transplant unit was recorded. The ideal num- There were more men with seborrhoeic dermatitis (86%) than ber of dermatology outpatient follow-ups was calculated. The without (59%). None had coexisting illnesses such as HIV or number of excisions required and prescriptions dispensed over Parkinson’s disease, with which seborrhoeic dermatitis may be a 1-year period was recorded. The national tariff was used to associated; 75% had other cutaneous findings including calculate the cost of outpatient follow-up and surgical excision seborrhoeic warts, xerosis and pityrosporum folliculitis. Sun of suspicious lesions and the cost of prescriptions was also exposure, both occupational and recreational, was more com- estimated. The records of 308 patients were reviewed. Sixty- mon in RTRs with seborrhoeic dermatitis than those without. one required 3-monthly follow-up for squamous cell carci- Only 38% recalled any exacerbating factors but sunshine was noma or malignant melanoma, 33 required 6-monthly follow- reported by over a third of this group. The majority reported up because of previous basal cell carcinoma, squamous cell mild-moderate disease severity (86%) and intermittent symp- carcinoma in situ or actinic keratoses, while the 191 who had toms (59%). The scalp and face were involved in over 80%, been transplanted for over 1 year would ideally be seen annu- the trunk in over 50% and flexures in over 20%. Erythema, ally. Follow-up outpatient appointments cost £46Æ81 leading scaling and greasiness of affected sites was mild-moderate in to a total of £23 451Æ81 for outpatient review alone. A total the majority. Severe seborrhoeic dermatitis was seen in 7%. of 155 minor operations were carried out over the study per- Many previous studies looking at cutaneous events in RTRs iod costing on average £757Æ60 each leading to a total of have not mentioned seborrhoeic dermatitis, while others have £117 428. Cryotherapy, 5-fluorouracil and imiquimod were reported low prevalence (4%). The higher prevalence of se- also required in 60 RTRs with an approximate cost of £16 borrhoeic dermatitis in our immunosuppressed group supports 000. The total cost was almost £160 000 for a population of the theory that infection is important in the aetiology – poss- 308 RTRs from a total local population of 1 160 000. This ibly with the Malassezia yeast. This theory is also supported by results in an annual average skin cancer management cost of the observed association between seborrhoeic dermatitis and £509Æ35 per RTR. RTRs require specialized dermatology fol- pityrosporum folliculitis, which is caused by Malassezia. Our low-up post-transplantation. Their burden of skin disease will study also raises the possibility of sun exposure being import- continue to increase as survival carries on improving. The ant in the pathogenesis of seborrhoeic dermatitis but further

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 65 multicentre studies would be required to fully investigate P-98 aetiological factors. Audit of azathioprine prescribing and monitoring in dermatology and other specialities A. Alkali, W. Myint and R.M. Azurdia Department of Dermatology, Broadgreen Hospital, Liverpool, U.K. Azathioprine is an immunosuppressive drug that is used in a P-96 range of dermatological conditions that are difficult to control Pain control in pyoderma gangrenosum using locally applied morphine as well as having many other uses across the specialities. S. Barker and M. Duffill* Adverse drug reactions to azathioprine are reported to occur Leeds General Infirmary, Leeds, U.K.; and *Waikato Hospital, Hamilton, in 15–28% of patients. The previously published BAD Guide- New Zealand lines (2004) included recommendations for routine safety Morphine is a commonly used analgesic for severe pain and monitoring of patients treated with azathioprine, especially when administered is usually given systemically. The mechan- pretreatment assessment of red cell thiopurine methyltransf- ism of action is via opioid receptors in the central nervous erase (TPMT) activity. A dermatology casenote audit of system. However, opioid receptors have also been detected on azathioprine prescribing and monitoring was undertaken peripheral nerve terminals in inflamed tissues. Several case between November 2004 and November 2005. A previous reports and two pilot studies have demonstrated reduction in local azathioprine guideline (2000) and the BAD guideline pain when opiates are applied locally to painful malignant were used as our standards of care for this audit. Patients pre- ulcers and to decubitus pressure ulcers. The mechanism of scribed azathioprine were identified using a patient database. action is thought to be via effects on the peripheral opioid A proforma was used to collect information from case notes. receptors. We report a case of pyoderma gangrenosum affect- Additionally, a questionnaire was circulated to all regional der- ing the breast in a patient with rheumatoid arthritis. Adequate matologists and to specialists in gastroenterology, haematol- pain relief was not obtained with systemic analgesics. Mor- ogy, rheumatology, nephrology, and transplant surgery – all phine sulphate 10 mg in 1 ml was mixed into 8 g of amor- regular prescribers of azathioprine. In total, 56 patients were phous hydrogel and applied at dressing changes to the ulcer. identified, taking azathioprine for a variety of conditions This resulted in a large reduction in pain and allowed lower including atopic eczema (33), psoriasiform eczema (6), hand doses of systemic analgesics. Topically applied opiates may eczema (4), photoaggravated eczema (2), bullous pemphigoid have a role in pain relief for pyoderma gangrenosum and (4), pemphigus vulgaris (5), lupus erythematosus (1), and other inflamed ulcerated skin conditions. (1). The questionnaire response rate was 54 of 84 (63%) with differing response rates across the specialties. From the case notes, before azathioprine treatment, all patients had screening blood tests (full blood count, urea and electro- P-97 lytes, and liver function tests [FBC/U&E/LFT]) performed and Topical pimecrolimus in – a preliminary study recorded. Pretreatment TPMT was performed in 44 of 56 but A.J. Kanwar this represented 100% testing of patients commenced on PGIMER, Chandigarh, India azathioprine after 2000; TPMT results were 41 of 44 (nor- Several therapeutic options are available for the treatment of mal), 3 of 44 (carrier). A patient information leaflet was vitiligo but none is uniformly effective. Pimecrolimus, which documented as being provided in 29% of patients. From the inhibits calcineurin, has recently been shown to be effective questionnaire, pretreatment TPMT was requested by 29 of 30 for the treatment of vitiligo. To evaluate the role of topical dermatologists, 4 of 9 nephrologists, 6 of 9 gastroenterolo- pimecrolimus 1% in the treatment of vitiligo, we conducted gists, 1 of 2 rheumatologists, and none of the transplant sur- this study. We enrolled 22 patients with limited vitiligo geons nor haematologists. The most common starting dose of lesions. Patients were asked to apply pimecrolimus 1% oint- azathioprine was an empirical dose of 50 mg daily. Only 16 ment twice daily. Repigmentation was recorded as minimal of 54 (30%) of all specialists prescribe azathioprine according (25%), moderate (50%), marked (75%) or complete (100%) to body weight. From the case notes, monitoring of FBC/ depending upon the extent of repigmentation in the treated U&E/LFT in the first month was as follows: weekly (25 lesions. Patients were followed up at monthly intervals for 4 patients), fortnightly (24 patients), and once (7 patients); months. Twenty patients could be evaluated at the end of 4 after the first month as follows: monthly (44 patients), two months as two patients left the study because of reasons monthly (6 patients), and three monthly (6 patients). Across unrelated to the study. At the end of treatment 12 patients the specialities there were 20 recorded episodes of leucopenia showed marked to complete repigmentation and four but no significant myelosuppression leading to cessation of patients showed moderate repigmentation. The remaining four azathioprine was reported. We conclude that since the previ- patients showed none to minimal repigmentation. Diffuse ous audit, all dermatology patients had pretreatment TPMT repigmentation was seen in the majority of the lesions. Our and baseline investigations before commencing azathioprine. results are encouraging and offer a new and potentially safe The starting dose of azathioprine in all patients was not based and efficacious treatment for this pigmentation disorder. on body weight as is recommended. Less than 50% of patients

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 66 Bristol Cup Posters had weekly FBC/U&E/LFT in the first month of therapy as is inflammatory effect, regulates B and T cells and inhibits com- recommended. Subsequent monitoring of therapy in dermatol- plement-mediated damage (Jolles S, Hughes J, Whittaker S. ogy, was done more frequently than is recommended in 89% Dermatological uses of high-dose intravenous immunoglobu- of patients. Awareness of the significance of measuring TPMT lin. Arch Dermatol 1998; 134: 80–6) These modes of action may across some specialties is patchy but from this audit it is diffi- account for its efficacy in the treatment of NL. The potential cult to know if TPMT testing has avoided the problems of use of IVIG in ulcerated NL requires further investigation. myelosuppression as no significant cases were reported during treatment. A larger, multi-speciality casenote audit of azathio- prine use would be useful to gain a clearer understanding of the usefulness of TPMT testing at baseline. P-100 What do we know about the safety of topical corticosteroids in pregnancy? P-99 C-C. Chi, C-W. Lee,* F.T. Wojnarowska and Improvement of ulcerated with G. Kirtschig intravenous immunoglobulin Nuffield Department of Clinical Medicine, University of Oxford, Oxford, J. Batchelor and P.M. Todd U.K.; *National Collaborating Centre for Women’s and Children’s Health, Addenbrooke’s Hospital, Cambridge, U.K. London, U.K.; Department of Dermatology, Churchill Hospital, Oxford, Necrobiosis lipoidica (NL) is a chronic granulomatous derma- U.K.; and Department of Dermatology, VU Medisch Centrum, Amsterdam, titis that usually arises on the shins. Severe, ulcerated cases of the Netherlands NL can be resistant to treatment. We report a case of ulcerated Topical corticosteroids are indicated for pregnant women with NL who responded well to treatment with intravenous im- pre-existing skin conditions or specific dermatoses of preg- munoglobulin (IVIG). A 40-year-old female patient presented nancy. We performed a systemic review of the safety of top- in 1991 with two erythematous, indurated lesions on the left ical corticosteroids used during pregnancy. We searched leg. She had a 20-year history of type I diabetes mellitus. The CENTRAL, MEDLINE, EMBASE, CINAHL, British Nursing Index lesions were diagnosed clinically as necrobiosis lipoidica (NL) and Archive, and Conference Papers Index. The types of stud- and treated with potent topical corticosteroid cream and ster- ies included were randomized controlled trials, cohort and oid-impregnated tape. In 2002, following an injury to the left case-control studies. Two reviewers independently screened shin, the NL plaque on her left leg flared and became painful. the search results and critically appraised the included studies. Treatment with topical tacrolimus and PUVA proved unsuc- We identified one relevant cohort and four case-control stud- cessful. The NL became ulcerated, painful, sloughy and infec- ies. Meta-analysis was improper due to considerable methodo- ted, requiring repeated courses of oral antibiotics. Oral logical heterogeneity, e.g. the settings ranging from pentoxifylline was contraindicated due to a previous history of multinational project to one-country population-based registry retinal haemorrhages. Oral hydroxychloroquine was com- to single teaching hospital, and the method of exposure ascer- menced but had to be discontinued owing to development of tainment varying from self-report questionnaire to database urticaria and angio-oedema. The ulcerated NL became infected analysis. A population-based case-control study revealed an with Pseudomonas and the patient was treated with meropenem, association between cleft lip ± palate and maternal corticoster- prednisolone, nicotinamide and lymecycline, plus larval ther- oid ointment use in the whole pregnancy [adjusted odds ratio apy to the sloughy areas. However, the lesions remained pain- (OR) 2Æ21, 95% confidence interval (95% CI) 1Æ11–4Æ39] and ful, ulcerated and highly debilitating. Treatment with in the first month of gestation [4.19 (1Æ47–11Æ97)], but not ciclosporin was contraindicated because of diabetic kidney dis- in the second and third months, the critical period for con- ease. Given the intractable nature of the NL, a 5-day trial of genital abnormalities. One case-control study based on a na- intravenous immunoglobulin (IVIG) 2 mg kg–1, was com- tional registry found no significant linkage between topical menced. Two weeks later, the ulcerated areas of NL had corticosteroid use in the first trimester and orofacial clefts improved dramatically. The patient also reported complete [adjusted relative risk 2Æ01 (95% CI 0Æ55–5Æ15)]. A multina- resolution of pain. Further improvement was seen four weeks tional case-control study showed no correlation between first later. NL is often a very difficult condition to manage. Initial trimester exposure to topical corticosteroids and cleft palate or treatment usually involves topical or intralesional corticoster- lip [OR 0Æ52 (95% CI 0Æ16–1Æ64)], cleft palate [0 (0–3Æ41)], oids. Further therapies include those mentioned above, and cleft lip ± palate [0.73 (0Æ23–2Æ37)]. However, this database also mycophenolate mofetil and anti-TNF agents. The patho- merely collected malformed infants with a history of maternal genesis of NL involves various immune and inflammatory first trimester drug exposure, and the controls were infants mechanisms including abnormal collagen that initiates inflam- with other congenital anomalies. Another population-based mation and recruitment of T cells, and deposition of immune retrospective cohort study illustrated that the adjusted ORs for complexes in vessel walls, suggesting a vasculitic process. Intra- low birth weight, malformations and preterm delivery among venous immunoglobulin modulates the release of certain cy- women receiving topical weak or medium potency cortico- tokines and cytokine antagonists, with an overall anti- steroids were 0Æ7(0Æ17–2Æ85), 0Æ93 (0Æ23–3Æ80) and 1Æ04

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 67

(0Æ56–1Æ92), respectively, and those for topical strong or very propionate 0Æ05% ointment alone was used in three strong corticosteroids were 1Æ23 (0Æ45–3Æ37), 0Æ56 (0Æ14– cases. Overall 27 of 34 patients benefited from the applica- 2Æ28) and 0Æ99 (0Æ54–1Æ84), respectively. Only one single- tion of topical corticosteroid, 24 of 27 had a >50% reduction hospital case-control study of 48 cases with nonsyndromic in pain, 24 of 28 had a >50% healing response, 2 of 2 had a cleft lip or palate found a significant increase in prevalence of good reduction in hypergranulation and 6 of 7 had a good maternal use of topical corticosteroids in the 1st trimester reduction in exudates. Only two wounds deteriorated and compared to 58 controls [adjusted OR 18Æ6(1Æ29–270)]. In treatment in these patients was immediately withdrawn. This conclusion, current available data considering the safety of study shows that application of topical corticosteroid to the topical corticosteroids during pregnancy is limited and mainly wound can accelerate healing and reduce pain in certain cases. related to fetal outcomes, which suggest a lack of association This implies that suppression of inflammation has an import- between them and oral clefts, low birth weight or preterm ant role to play in healing and pain relief. However, careful delivery. However, the findings are inconclusive, and more monitoring of wounds is essential as there is a risk of sensiti- robust large-scale study is required. zation or delayed healing. Future case-control studies could look in more detail at the use of corticosteroids in chronic wounds. P-101 The role of topical corticosteroids in the management of chronic leg ulcers P-102 D. Hofman, T. McPherson, R. Cooper,* M. Eagle, Treatment of erythema multiforme major with K. Moore and S. Cooper thalidomide: a report of two cases Oxford Dept Dermatology Churchill Hospital, ORH Trust, Oxford, U.K.; C. Leitch and C. Jury *Blackwater Valley, Hart, U.K. and North Hants Trust, North Hants, Glasgow Royal Infirmary, Glasgow, U.K. U.K. Erythema multiforme major (EMM) has recently emerged as To date there are no published prospective studies of topical a distinct clinical disorder characterized by mucosal erosions corticosteroid use for chronic leg ulcers. Despite limited exist- plus typical or raised atypical target lesions in an acral and/ ing evidence topical corticosteroids are widely used in the or facial distribution. It is thought to be induced mainly, management of chronic wounds. In theory corticosteroids although not exclusively, by herpes virus infection, distin- could have a role in wound healing by suppressing chronic guishing it from Stevens–Johnson syndrome which is inflammatory responses to accelerate healing and reduce pain. thought to be solely a drug-induced phenomenon. Thalido- However, they may also delay skin healing and could increase mide has been used to treat many refractory dermatological the risk of infection. The aim of this study was to evaluate the conditions (Wu JJ, Huang DB, Pang KR et al. Thalidomide: use of topical steroids in a cohort of patients with leg ulcers dermatological indications, mechanisms of action and side- and to document the response to treatment. This observational effects. Br J Dermatol 2005; 153: 254–73). The successful study identified current practice in three specialist wound treatment of erythema multiforme with thalidomide was healing clinics. Over three months, consecutive leg ulcers first reported in 1982 in a single case report and this has selected for topical corticosteroid treatment were recorded. been followed in the European literature by six other case The aetiology of the leg ulcer, the indication for corticosteroid reports and one retrospective study involving 26 patients. treatment, the corticosteroid used and the efficacy of treatment We would like to present two cases of successful treatment were recorded. Data on healing, pain relief, exudate reduction of EMM with thalidomide in the U.K. The first case is that and control of hypergranulation tissue was collected. The heal- of a 33-year-old woman who has been troubled by fre- ing response was graded as reduction in surface area. Levels of quent, disabling attacks of oral and genital ulceration diag- pain experienced by patients were assessed by patient-rated nosed as EMM in 2000. Previous treatment has included visual analogue scales. Overgranulation tissue and control of acyclovir, famciclovir, azathioprine, dapsone, intravenous exudate effects were rated as none, poor, good, or total. Data immunoglobulin, ciclosporin, hydroxychloroquine and virtu- on 34 patients were recorded. The most frequent ulcerations ally continuous prednisolone over the last six years, titrated were venous (8), arterial (2) and mixed (5). Other aetiologies to disease activity. She has had no significant periods of included rheumatoid arthritis, vasculitis and eosinophilic fasci- remission, experiencing attacks every six to eight weeks and itis. The most frequent indications for treatment were pain requiring several admissions to hospital. After commencing control (27) and stimulation of healing (26). Other indica- thalidomide 50 mg daily in July 2006 she had rapid resolu- tions cited were reduction of granulation (2) and control of tion of her skin lesions. She is being maintained on 50 mg exudates. In most cases more than one reason was given. The thalidomide 3 times per week and has had no relapses in most frequently used applications were combined corticoster- the six months to date. The second case is that of a 50- oid, antibiotic, antifungal preparations, clobetasol propionate year-old woman who was diagnosed with EMM in 2001. 0Æ05% with neomycin sulphate 0Æ5% and nystatin 100 000 She has found the condition so disabling that she has been units g–1 (17) and clobetasone butyrate 0Æ05% with oxytetra- unable to work for long periods. Her previous treatments cycline 3% and nystatin 100 000 units g–1 (13). Clobetasol have included dapsone, topical tacrolimus, ciclosporin,

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 68 Bristol Cup Posters mycophenolate mofetil, acyclovir, famciclovir, azathioprine, P-104 colchicine and again, virtually continuous prednisolone titrated A combined photodynamic therapy, imiquimod, to disease activity. She has had no significant periods of curettage and cautery approach in the treatment of remission. She commenced thalidomide 50 mg per day in large recalcitrant basal cell carcinomas December 2006 and to date has had a good response with M. O’Connell, M. Walker, D. Scott* and A.M. Layton resolution of lesions and no relapses. These two cases high- Department of Dermatology, Harrogate and District NHS Foundation Trust, light the role for thalidomide in the treatment of refractory Harrogate, North Yorkshire, U.K. and *Dept. of Histopathology, Harrogate EMM. Prescription of thalidomide in the U.K. currently and District NHS Foundation Trust, Harrogate, North Yorkshire, U.K. requires the registering of the prescriber, pharmacist and Photodynamic therapy (PDT) is a well established treatment patient, and adherence to the System for Thalidomide for a range of pre-malignant and malignant skin lesions Education and Prescribing Safety (STEPS). Due to the well which includes actinic keratoses and basal cell carcinomas documented side-effect of neuropathy, regular nerve con- (BCCs). The major benefits of this approach are that it is duction studies are also recommended. noninvasive and often leads to excellent cosmetic results post-treatment (Vinciullo C, Elliott T, Francis D et al. Photo- dynamic therapy with topical methyl aminolaevulinate for P-103 ‘difficult-to-treat’ basal cell carcinoma. Br J Dermatol 2005; Management of chondrodermatitis nodularis helicis 152: 765–72). We describe two cases of large (>10 cm) by auricular pressure-relieving device: a BCCs in two male patients. Both presented with enlarging retrospective study BCCs on the back and the diagnosis of superficial BCC was A. Belgi and R.A. Logan* confirmed by biopsy in both cases. The lesions were felt to Welsh Institute of Dermatology, University Hospital of Wales, Cardiff, U.K. be too extensive for radiotherapy. Initially, topical fluoroura- and *Princess of Wales Hospital, Bridgend, U.K. cil cream was administered to both lesions twice daily for We have conducted a retrospective questionnaire study to four weeks. At the end of the treatment period, neither determine the efficacy in our hands of an auricular pres- patient demonstrated full resolution of the lesion. Imiqui- sure-relieving device in the management of chondroderma- mod 5% cream was then applied for five days of each titis nodularis helicis (CNH - painful nodule of the ear). week for six weeks applying the cream directly to the le- Over the last 10 years 78 patients have been treated using sion and 1 cm beyond it. Response at 12 weeks demonstra- a custom-made device made by our maxillo-facial surgery ted that both lesions were still active with some nodular technicians using an established manufacturing protocol. The areas of BCC being identified from repeat biopsies. PDT was completed questionnaire was returned by 48 patients (24 then administered on two separate occasions two weeks males, 24 females), age range 41–86 years. Only seven apart using methyl aminolaevulinate 160 mg g-1 followed patients (15%) found the prosthesis effective in relieving by exposure to red light (570–670 nm) at 75 J cm-2.At their symptoms; 20 patients (42%) did not get any symp- follow-up one month post-treatment, there were still resid- tomatic relief while 20 (42%) reported only partial relief. ual areas of BCC. As the size of these lesions represented a Thirty-three patients (70%) had difficulty tolerating the significant surgical challenge, it was decided to continue device for various reasons but only 4 (8%) stopped using it with PDT in combination with imiquimod over the course as a result. Those who found the cushion effective contin- of the next six months. The patients used topical imiqui- ued to use it for periods ranging from 1 to 6 months mod for five days of every week for four weeks. After a (median 6 months). In contrast those for whom the device further two weeks, the patients received a course of PDT. was either partially effective or ineffective persevered with This treatment regime was repeated on six occasions. The it for periods ranging from 1 day to 6 months (median 3 patients have now been clear of disease for 24 months. months). In contrast to previous reports [Pereira JA, Allen Although PDT is predominately used in superficial BCCs, it DL, Woollons A et al. Auricular pressure relieving device for can prove very effective in the treatment of nodular BCCs the treatment of chondrodermatitis nodularis helicis chroni- (Rhodes LE, De Rie M, Enstrom Y et al. Photodynamic ther- ca. Br J Dermatol 1999; 141 (Suppl. 55): 75 (Abstract); apy using topical methyl aminolaevulinate vs. surgery for Moncrieff M, Sassoon EM. Effective treatment of chondro- nodular basal cell carcinoma: results of a multicenter ran- dermatitis nodularis chronica helices using a conservative domized prospective trial. Arch Dermatol 2004; 140: 17–23). approach. Br J Dermatol 2004: 150: 892–4], we have found These cases illustrate how combining PDT with imiquimod that the use of a pressure-relieving device was not univer- can lead to an increased therapeutic effect in the manage- sally effective in managing CNH. The reasons for this were ment of large BCCs. It also highlights how multiple treat- mainly technical, such as difficulty applying the device, dis- ments may be required but it is well tolerated and gives an comfort due to bulkiness, poor retention and tearing. excellent cosmetic outcome.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 69

P-105 dermatitis and response to dapsone. Clin Exp Dermatol 1989; 14: Audit of ultraviolet B radiation desensitization in the 382–4). We report a case of FM of nearly 18 years duration treatment of polymorphic light eruption which has undergone complete remission with dapsone. A C. Feighery and K. McKenna 52-year-old lady presented to us with history of recurrence of Belfast City Hospital, Belfast, U.K. an itchy rash on her back, arms, breast and scalp for 4 The primary management of polymorphic light eruption (PLE) months. Lesions on her scalp were associated with hair loss. involves avoidance of and protection from exposure to ultravi- She gave a past history of long standing pruritic rash on and olet radiation (UVR). In a minority of patients these measures off for nearly 18 years; two previous biopsies showed nonspe- are not sufficient and symptoms through the summer months cific histology. She responded poorly to topical steroids, UVB can be severe and unbearable. In these types of patients artifi- and PUVA phototherapy but it cleared completely with cial ‘hardening’ of the skin by low dose UVR treatment can 150 mg of dapsone daily for 9 months. The rash recurred be effective in improving the symptoms. We aimed to exam- after 4 years in January 2005. Examination showed skin le- ine methods, side-effects and the success of this type of treat- sions suggestive of cutaneous T-cell lymphoma (CTCL) on her ment in patients being treated in a tertiary referral centre from trunk, arms and breast. She also had two tumid scaly and 2003–2005. We examined the charts of patients who had shiny hairless plaques on the scalp. The systemic examination desensitization treatment from 2003–2004 and included any and routine investigation were normal. A biopsy from the treatments these patients may have had in the years before scalp showed well developed mucinous degeneration of the this. Charts were identified from a database held in the depart- follicular epithelium with accumulation of mucin centrally. ment from 2003. We gathered information from the charts There was no evidence of CTCL on histology. Topical steroids including patient sex, diagnosis and number of treatments. and tacrolimus were ineffective. She was commenced on oral We examined each treatment session looking at a protocol of PUVA but her rash deteriorated. Dapsone in the dose of desensitization, diagnostic tests, and reported result. We iden- 100 mg daily was considered as she had improved with it in tified 23 patients who had had 43 treatment sessions in total. the past. After 8 weeks the rash cleared and at 6 months there The majority of these patients were female (20), 15 had had was a complete regrowth of hair. Treatment was stopped after treatment in March and 19 had had treatment in April. PLE 9 months. There is no recurrence at 9 month follow-up. was provoked in 11 patients. The response was reported as excellent in two and good in 26. Overall the results were good and although PLE was provoked in almost half of our patients this is comparable with larger trials (Man I, Dawe RS, P-107 Ferguson J. Artificial hardening for polymorphic light erup- The treatment of calciphylaxis with intravenous tion: Practical points from ten years’ experience. Photodermatol sodium thiosulphate Photoimmunol Photomed 1999; 15: 96–9). R. Rose, B. Walker and C. Wilson The General Infirmary at Leeds, Leeds, U.K. Calciphylaxis is a life-threatening complication of renal disease P-106 which is increasing in incidence. It is difficult to treat, not Successful treatment of follicular mucinosis with least because the pathogenesis is poorly understood. A recently dapsone introduced therapy is intravenous sodium thiosulphate – the T. Desai and S.B. Bittiner rationale being its successful use in nephrolithiasis and tumor- Doncaster & Bassatlaw Hospitals NHS Foundation Trust, Doncaster, South al calcinosis (Brucculeri M, Cheigh J, Bauer G, Serur D. Long- Yorkshire, U.K. term intravenous sodium thiosulfate in the treatment of a Follicular mucinosis is a condition characterized by accumula- patient with calciphylaxis. Seminars in Dialysis 2005; 18: 431–4). tion of mucin in hair follicles. It occurs in various inflamma- The suggested mechanism is that it acts as a calcium chelator tory, infectious and neoplastic skin conditions. After its but it also appears to have antioxidant properties. A 39-year- initial description by Hermann Pinkus, a classification was old male was admitted with nephrotic syndrome secondary to proposed to describe two main types of follicular mucinosis diabetic nephropathy. Following the onset of severe peripheral (FM). The first type occurs in young adults, in absence of any oedema he developed bilateral painful leg ulceration. Other other pathology and resolves spontaneously. The second type medical history included type 2 diabetes mellitus and dilated occurs in elderly patients and is associated with mycosis fun- cardiomyopathy and medications comprised warfarin, doxazo- goides and Se´zary syndrome. However, several long term fol- sin, carvedilol, furosemide and metformin. On examination low-up studies and clinicopathological reviews suggest the there were extensive areas of woody induration and necrosis two types of FM represent a single disease entity with a vari- affecting both thighs and lower legs. Relevant investigations able spectrum; those with the benign form representing indo- included creatinine 307 lmol L–1, adjusted calcium 2Æ21 lent forms of cutaneous T-cell lymphoma. Treatment of mmol L–1, phosphate 1Æ95 mmol L–1, parathyroid hormone chronic, persistent disease can be difficult and dapsone is 23Æ6 pmol L–1 and elevated anticardiolipin IgG antibodies. reported to be useful in occasional cases (Rustin MH, Bunker Arterial duplex imaging showed evidence of calcification of CB, Levene GM. Follicular mucinosis presenting as acute the distal superficial femoral arteries, and a skin biopsy

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 70 Bristol Cup Posters showed extensive calcification of capillary and artery walls in trexate was discontinued and etanercept 50 mg was given the subcutaneous fat, with chronic inflammation and fat subcutaneously twice weekly in combination with deltacortril necrosis. Warfarin was discontinued and subcutaneous heparin 5–10 mg resulting in a 30% reduction in induration which commenced. An oral phosphate binder was introduced which was not sustained four months later. Therefore adalimumab corrected the phosphate and parathyroid hormone levels. He 40 mg s.c. weekly was substituted in combination with pred- was treated with diuretics and a number of desloughing nisolone 5 or 10 mg on alternate days. After 6 months there agents and light compression. Over the ensuing 2 months the is a significant reduction in erythema and induration. Nonca- lesions steadily progressed proximally and hence he com- seating granulomas are composed of macrophages and CD4+ menced intravenous sodium thiosulphate at a dose of 25 g, TH lymphocytes which secrete interleukin (IL)-12, interferon administered 3 times per week. This was well tolerated and c and tumour necrosis factor (TNF) a which direct naı¨ve T had a marked effect within a month, resulting in cessation of lymphocytes to differentiate into activated TH cells (Moller the disease progression and gradual healing of the more prox- DR. Treatment of sarcoidosis – from a basic science point of imal lesions. Unfortunately he developed MRSA septicaemia view. J Intern Med 2003; 253: 31–40). Increased levels of TNF and subsequently became dialysis-dependent. He then went alpha have been demonstrated in alveolar macrophages of on to develop new ischaemic changes to his feet, which ulti- patients with pulmonary sarcoidosis (Baughman RP, Strohofer mately required bilateral below knee amputations. Whilst the SA, Buchsbaum J, Lower EE. Release of tumour necrosis factor outcome in this case was influenced by co-morbidity, the ini- by alveolar macrophages of patients with sarcoidosis. J Lab Clin tial response was dramatic in a condition where other treat- Med 1990; 115: 36–42). Cutaneous sarcoidosis may behave in- ments are essentially lacking. In the few reported cases of dependently of systemic sarcoidosis and prove difficult to patients treated with sodium thiosulphate, infusions are often manage. Adalimumab proved effective in our case. Authors required for many months to allow healing. However as this have no financial interest drug is relatively well tolerated, it should be considered a valuable therapeutic tool in this distressing condition. P-109 An audit of acitretin prescribing: the need for clinical P-108 guidelines Recalcitrant cutaneous sarcoidosis responds to M. Roche and K.E. McKenna adalimumab but not to etanercept Belfast City Hospital, Belfast, U.K. S. Field, M.X. Fitzgerald, K. Sheahan and P. Collins Acitretin is a retinoid which is licensed for severe extensive St. Vincent’s University Hospital, Dublin, Ireland psoriasis, palmo-plantar pustular psoriasis, Darier’s disease and A 43-year-old female with an 18-month history of biopsy- severe congenital ichthyosis. The side effects are manifold and proven cutaneous sarcoidosis was referred in August 2004. include teratogenicity, dryness of skin and mucous mem- Plaques developed on her elbow and the distal half of her branes, and musculoskeletal effects. Currently, no acitretin nose; these gradually extended to her upper lip. She noted prescribing or monitoring guidelines exist. We performed an swelling of her nose and occasionally soreness and itching. audit of acitretin prescribing in our dermatology unit in order Treatment with hydroxychloroquine 400 mg alternating with to assess the numbers of patients on acitretin, to assess investi- 200 mg daily, minocycline 100 mg daily and moderately po- gations performed before and during treatment and their fre- tent topical steroid was unsatisfactory. Four years earlier she quency, and to assess side effects experienced or documented. was diagnosed with pulmonary sarcoidosis after presenting Acitretin was prescribed over a two year period for 37 patients with . This was stable and had improved attending our department. Of these, 54% were male and 46% without treatment. She had no other significant medical or were female. The average patient age was 60 years, with a family history. Examination showed an annular erythematous range of 30–89 years. Ten of these patients had previously plaque on the distal half of her nose extending onto her upper received etretinate. The indications for treatment were varied: lip, a 1 cm plaque over the left zygoma, a 2 cm plaque on 51% had chronic plaque psoriasis, 19% had had multiple non- her left elbow and two nodules, 0Æ5 cm each, on the right melanoma skin cancers, 19% had palmo-plantar pustular arm. Full blood count, renal, liver, bone profile and angioten- psoriasis, and 11% had Darier’s disease. No side effects were sin converting enzyme level were normal. The chest X-ray documented in the notes for 70% of the patients. Documented showed bihilar lymphadenopathy. Pulmonary function tests side effects were noted in 30% and included myalgia, dry skin and diffusing lung capacity with carbon monoxide were nor- or lips, ocular effects and nail changes. Therapy had to be dis- mal. The initial treatment comprised prednisolone 40 mg daily continued by 13 patients (35%), either because of intolerable for 8 weeks and then a gradual reduction to 10 mg daily, side effects, abnormal investigations or lack of clinical hydroxychloroquine 400 or 200 mg on alternate days and response. The duration of treatment ranged from two weeks methotrexate 15–17Æ5 mg once weekly for one year. Initial to 24 years. Investigations were performed monthly for two improvement was not sustained. One course of intralesional months and three monthly thereafter in most of the patients. triamcinolone was complicated by facial cellulitis. Repeat skin A full blood count before and on treatment was performed in biopsy from the nasal plaque showed active disease. Metho- 89% of patients, and no abnormalities related to treatment

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 71 were noted. Liver and lipid profiles were performed in 97% 6-mercaptopurine from which she had no benefit despite repea- and 84% respectively. Elevated cholesterol levels developed on ted hospital admission. Associated side-effects on these treat- treatment in 41% of patients, and 51% developed elevated tri- ments included diarrhoea with azathioprine and hypertension glyceride levels. Pre-treatment X-rays of wrist, ankle or spine with ciclosporin. Repeated skin biopsies and elevated IgE levels were performed in 41% of patients. X-rays were repeated on confirmed the diagnosis of chronic atopic eczema. She was treatment in 38% of patients, but only one patient developed commenced on IVIG (Flebogamma5%) 0Æ4mgkg–1 day–1 a bony abnormality presumed to be caused by acitretin. This for 5 days plus potent topical steroids. She responded well audit highlights the variability amongst clinicians in perform- and her erythroderma resolved without significant side-effects ing pre- and on-treatment investigations in patients on acitre- within 2 weeks of commencing this treatment. She has tin. This problem is compounded by a lack of available required four additional admissions to hospital for treatment clinical guidelines. Our data show that the need for perform- of erythrodermic episodes with IVIG and achieves remission ing full blood count on treatment would appear to be limited. for approximately 3–4 months between treatments. This case Abnormalities in lipid profiles were the commonest objective series highlights IVIG as a suitable alternative for the treatment side effect of treatment. Regular X-ray imaging appeared to be of recalcitrant atopic eczema with the additional benefit of unnecessary in those patients without musculoskeletal symp- good tolerability relative to other systemic agents used to treat toms, and we would suggest that following baseline X-rays, atopic eczema. patients should only have further X-rays if they become symp- tomatic on treatment. P-111 Patch testing to aeroallergens, especially house P-110 dust mite, is often positive in atopics with facial or Intravenous immunoglobulin for the treatment of hand dermatitis severe, recalcitrant atopic eczema in the elderly N. Hallai and D.J. Gawkrodger J. Fallon and G.M. Murphy Royal Hallamshire Hospital, Sheffield, U.K. Beaumont Hospital, Dublin, Ireland Positive patch tests to house dust mite (HDM) are found in Recalcitrant atopic eczema may require systemic treatments for atopic dermatitis (Goon A, Leow Y-H, Chan Y-H et al. Atopy control; however these agents, particularly immunomodulato- patch testing with aeroallergens in patients with atopic derma- ry drugs, often have significant side-effects, particularly in the titis and controls in Singapore. Clin Exp Dermatol 2005; 30: elderly. We report two cases of recalcitrant atopic eczema in 627–31), but the significance is unclear. In order to clarify elderly patients that responded successfully to treatment with the role of aeroallergens, and examine correlations between intravenous immunoglobulin (IVIG). A 75-year-old female immediate and delayed type allergy and atopic status, we with a 10-year history of widespread eczema failed to respond undertook a study of patch testing and specific IgE tests to to various topical treatments including emollients, steroids, aeroallergens, correlated with atopic status and clinical presen- tacrolimus, broadband ultraviolet (UV) B therapy, PUVA tation over 12 months. Atopic individuals, i.e. those with a phototherapy (nine sessions, cumulative dose 3Æ5Jcm–2) and personal history of asthma, eczema or hay fever, with either TL01 UVB therapy. She also failed to respond adequately to dermatitis in an airborne distribution and/or a history of dust systemic treatment regimes including steroids, ciclosporin allergy, were patch tested with an allergen series consisting of (200 mg daily), methotrexate (12.5 mg once weekly) and HDM 20% (50% Dermatophagoides farinae and 50% D. pteronyssinus) mycophenolate mofetil 1 g twice daily. Associated side-effects (Chemotechnique Diagnostics, Vellinge, Sweden), with the on these treatments included Cushing’s syndrome with following allergens supplied by Stallerpatch (Stallergenes SA, steroids, and hypertension with ciclosporin. Skin biopsies Freˆnes, France): mixed grasses, birch, D. farinae, D.pteronyssinus, and elevated IgE levels confirmed atopic eczema. Patch tests mugwort, cat, dog and plantain. A control group consisted of were negative. The patient was subsequently commenced on nonatopics with similar clinical features. Almost all patients IVIG (Flebogamma 5%, Grifols U.K. Ltd, Cambridge, U.K.) had allergen-specific IgE tests to aeroallergens. We evaluated 0Æ5mgkg–1 day–1 for 4 days without significant side-effects. 52 of 58 patients: 38 women and 15 men. Forty-four were in The patient had two further IVIG infusions at the same dosage the study group, 35 had dermatitis of the face, seven of the at one month intervals and within 1 month of the final dose a hands, one of the forearm and lower limbs and one the axil- marked improvement of the patient’s eczema was observed. lae. There were eight controls, all women with facial derma- Her eczema is now adequately maintained on daily topical titis. Of the study group, 32 exhibited at least a D4+ to HDM, emollients and tacrolimus 0Æ1% ointment which is intermit- regarded as of current relevance in 25 and of unknown rele- tently substituted with potent topical steroids for exacerba- vance in seven. Five of the 32 HDM positive patients were tions. A 74-year-old female with a 3-year history of atopic positive to D. farinae, five to D. pteronyssinus and two to both. eczema developed recurrent episodes of erythroderma. This Twenty-one had grade 2 or higher allergen-specific IgE to was despite multiple topical treatments including emollients, HDM, 10 had no antibodies and one was not tested. In the steroids, tacrolimus, broadband UVB therapy and subsequently study group 15 reported respiratory symptoms or deterior- the following systemic agents ciclosporin, azathioprine and ation of eczema after dust exposure. Two in the control

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 72 Bristol Cup Posters group, both of whom had reacted to dust but were not con- 400 yards away. Fitzroy Square was designed by Robert Adam sidered atopic, were positive for HDM on patch testing. One in 1794. The square’s southern and eastern sides, built in also had allergen-specific IgE to HDM. Reactions to other aero- Portland stone, remain in their original form. In 2003 the allergens were infrequent: one reacted to cat, one to dog Association had once again outgrown its accommodation and (both thought relevant), one to birch and one to grasses (both decided to move across the Square to the much more spacious of unknown relevance). We are presently examining follow- number 4, our new home for the BAD. In 2006, with major up data to see if HDM avoidance was beneficial. Overall, 32 renovations nearing completion, it was decided to bestow the atopic patients with facial or hand dermatitis had allergic posi- name Willan House on the Association’s new property, in tive patch tests to 20% HDM (Chemotechnique). Of those memory of Robert Willan. Dermatology is not new to Fitzroy with a positive patch test to HDM, 65% also demonstrated Square. In 1852 Fitzroy Square housed the Western Dispensary allergen-specific IgE. The relationship between immediate type for Diseases of the Skin, but the history of this is not well and cell-mediated allergy to HDM needs to be explored known. On the other hand, The London Skin Hospital, foun- (Rawle FC, Mitchell EB, Platts-Mills TA. T cell responses to the ded in 1887, at 40 Fitzroy Square West, remained functional major allergen from the house dust mite Dermatophagoides pteron- until 1948, when it changed into the Eastern portion of the yssinus, Antigen P1: comparison of patients with asthma, atopic Foot Hospital. dermatitis, and perennial rhinitis. J Immunol 1984; 133: 195– 201). However, it is too soon to recommend this as a stand- ard procedure since its significance is not fully determined. P-114 Sir Jonathan Hutchison: a clinician for all seasons D.J. Eedy Craigavon Area Hospital Group Trust, Portadown, U.K. P-112 Jonathan Hutchinson, a devout Quaker, was educated at Selby, Abstract withdrawn. Yorkshire, and qualified at Bartholomew’s Hospital, where he was influenced by Sir James Paget. Hutchinson became Sur- geon to the London Hospital and Moorfields Eye Hospital, P-113 (1862–1878). Initially a surgeon, he became interested in The homes of the British Association of dermatology through the influence of James Startin at the Dermatologists newly founded Blackfriars Skin Hospital. Once he was on the D.J. Eedy staff, Blackfriars Skin Hospital undoubtedly became the fore- Craigavon Area Hospital Group Trust, Portadown, U.K. most centre for dermatology and syphilology in this country In 1919, Sir Archibald Gray, the then Editor of the British and developed close contacts with the Hoˆpital St Louis, Paris. Journal of Dermatology suggested the formation of the British The list of first descriptions attributed to Hutchinson is Association of Dermatologists. He perceived that British Der- astounding. The best known is the Hutchinson melanotic matology lagged behind some of its European counterparts (1894), which maintains his eponymic fame, but the and believed that many provincial dermatologists felt disad- list also includes sarcoidosis, melanotic , Peutz–Jegher vantaged and the ‘poor relations’ of their London counter- syndrome (and its association with intussusception of the parts. He brought before the Editorial Committee in May bowel), arsenical keratosis, keratoacanthoma, angioma serpigi- 1921, the proposal that there should be an Association of Brit- nosum, cheiropompholyx and the apple jelly nodules of lupus ish Dermatologists. The first meeting proper then followed on vulgaris. He described Hutchinson’s teeth and pupils long the 18th of November 1921 at the Royal Society of Medicine. before Treponema pallidum or the Wassermann reaction were In 1965 Gray’s dream of a home for the Association was ful- recognized. He was elected President of the London Third filled by acquiring the Willan Room at the Royal College of International Dermatological Congress in 1896. His presence Physicians, which was used for meetings of our committee was the one redeeming feature of this international meeting, and executive. Until then administration had been done on which otherwise brought London little kudos, where he the dining room tables of the Honorary Secretaries. With the arranged an array of clinical cases. Hutchinson edited the Brit- coffers of the Association increasing, mainly from the Journal ish Medical Journal from 1877 to 1888, and produced the and the annual meetings, in 1979 the Association moved into two volumes of his Atlas Illustrating Clinical Surgery. He personally its first official Office, accommodated in the basement in John wrote 11 volumes of Archives of Clinical Surgery (in the Wil- Street, Blackwell’s London headquarters. This remained our lan library), from 1889 until 1900, to include the earliest home until 1986 when space became available in St Andrew’s description of sarcoidosis termed ‘lupus vulgaris multiplex Place. The first tenancy was in 1972 but as the first office was non-ulcerans’. Despite his brilliance he could be obstinate. less than satisfactory, in the following year we moved to the For example, he staunchly maintained that the cause of brighter and more spacious basement of 3 St Andrew’s Place. leprosy was the consumption of decaying fish and that urti- In 1994 the Association, having outgrown its basement caria pigmentosa was invariably due to bed bugs, even after accommodation in St Andrew’s Place, purchased the spacious their aetiology was known. Despite these flaws he was one of new high-profile headquarters at 19 Fitzroy Square, only some the great medical geniuses of his time and towards the end of

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Bristol Cup Posters 73 his career, spent more time at his ‘pleasure dome’ at the austere, seemingly humourless figure. In 1893 he opened, at Blackfriars Skin Hospital. Hutchinson’s amazingly retentive his own expense, a Clinical Museum at 1 Park Crescent, and memory, coupled with his genius for teaching, enabled him transferred it to a house in Chenies Street in 1898 which can to fascinate large audiences. With his tall, stooping frame, his be regarded as the forerunner of London’s Postgraduate Med- untidy black beard and his slow speech, he presented an ical School.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 23–73 Historical Archive Symposium: Summaries of Papers

H-1 management of cutaneous conditions, however; in more A concise history of the inorganic elements in the recent times, perhaps owing to the poor quality of evidence treatment of cutaneous conditions to support their use, many of these compounds have been left D.A. Thompson and A.G. Smith vulnerable to replacement by more expensive products. University Hospital of North Staffordshire, Stoke-on-Trent, U.K. Elements such as gold, silver, tin, copper, lead, iron and mer- cury have been known since antiquity but were almost never used in the pharmacology and medicine of the ancient world. The Romans favoured vegetable-based drugs, and Galen (c. AD 130–201), a pre-eminent physician of the Roman Empire, H-2 was a firm believer in botanical medicines and thought metals Bloodroot: an ancient remedy in modern times to be poisons. Inorganic elements in their various forms M. Kalavala and C. Mills entered Western medical practice during the Renaissance with Royal Gwent Hospital, Newport, Gwent, U.K. the work of the German-Swiss physician and alchemist, Para- Bloodroot (Sanguinaria canadensis) is a flowering plant native to celsus (1493–1541). Born Philippus Aureolus Theophrastus eastern North America. Bloodroot stores sap in an under- Bombastus Von Hohenheim, he gave himself the Latin name ground rhizome. The roots, usually used fresh, are made into of Paracelsus, meaning ‘‘greater than Celsus’’, referring to the washes, poultices, snuffs, dental powders, and escharotic sal- great encyclopaedist of the first century, Aulus Cornelius Cel- ves. Bloodroot was used by native Americans as a dye, as a sus. Paracelsus encouraged alchemists to ‘stop looking for herbal remedy for the treatment of cancer, moles, warts, and gold, instead find medicines’. He identified the active ingredi- as an emetic. Recently we saw an 18-year-old male patient ent in each remedy so that it could be extracted, purified and who developed scarring after two applications of Derma- given in a controlled dose for specific diseases, and thus estab- TendTM mole remover to three moles on his back. The active lished the role of chemistry in medicine. He introduced pow- ingredient listed in the preparation is North American Blood- dered tin as an antihelminthic, mercury compounds instead of root. No further details like concentration of active ingredient metallic mercury for , and developed treatments using and excipients are mentioned. The suppliers did not disclose antimony, arsenic, copper, iron, lead, sulphur and potassium any further information when contacted by e-mail. The appli- salts. He was the first to name the element zinc in 1526 and cation of bloodroot to the skin results in the formation of an introduced zinc and zinc salts to medicine, but it was Hennig eschar. Mohs’ chemosurgery used bloodroot, zinc chloride Brand who made the first scientific discovery of an element in and antimony trisulphite paste to fix tumour in tissue, fol- 1649 when he discovered phosphorus. The next two hundred lowed by surgical excision. Harry Hoxsey, the lay cancer spe- and twenty years would see the discovery of many more ele- cialist, promoted application of a paste similar in composition ments, and by 1869, when the Russian chemist Dimitri Iva- to Mohs’ paste to treat external cancers. In 1948 Mohs’ novich Mendeleev (1834–1907) published a periodic table of renounced the use of escharotics alone as they caused exces- elements, a total of sixty-three elements had been discovered. sive mutilation with unreliable cure (McDaniel S, Goldman The acceptance of inorganic elements as medicines increased GD. Consequences of using escharotic agents as primary treat- between the 16th and 18th centuries, and throughout the ment for nonmelanoma skin cancer. Arch Dermatol 2002; 138: 19th and 20th centuries their use became well established in 1593–6). Bloodroot produces benzylisoquinoline alkaloids, the pharmacopoeia. As a result, the field of dermatology has primarily sanguinarine. Sanguinarine has selective antiprolifer- benefited from their use both as topical and as systemic drugs. ative and apoptotic activity against human epidermoid carci- Mercury, arsenic bismuth and potassium iodide have all been noma cells (Ahmad N, Gupta S. Husain MM et al. Differential administered orally to treat syphilis, and hair tonics popular antiproliferative and apoptotic response of sanguinarine for both men and women in the 19th century often contained for cancer cells versus normal cells. Clin Cancer Res 2000; 6: lead compounds. Topically, mercury has been used as a cos- 1524–8). Natural and alternative remedies are increasingly metic bleaching agent while, aluminium and copper salts have used by patients to treat skin lesions. Many of these prepara- been used for their antifungal and astringent properties. Sul- tions are of unknown strength and purity with no scientifically phur has been prescribed for eczema, psoriasis, seborrhoeic documented proof of efficacy. It is now necessary to include dermatitis, fungal infections, acne and scabies. The inorganic an enquiry in to the use of such products when we assess skin elements have enjoyed a long history of use in the lesions.

2007 The Authors 74 Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 74–80 Historical Archive Symposium 75

Posters

H-3 H-4 Dr Robert Prosser White: a pioneer of occupational The three ages of epidermolysis bullosa: a brief dermatology history S. Ogden, D. Ramlogan* and E. Stewart* J. Hague, S. Orpin, R. Eady* and A.H. Heagerty Hope Hospital, Salford, U.K. and *Leigh Infirmary, Wigan, U.K. Heart of England NHS Foundation Trust, Solihull, West Midlands, U.K. and Dr Robert Prosser White (1855–1934) was renowned in his *St John’s Institute of Dermatology, London, U.K. hometown of Wigan as a certifying factory surgeon for the The defining of epidermolysis bullosa (EB) as we understand it surrounding industrial works (iron works and cotton mills), today can be divided in to three phases: clinical, ultrastructural yet few of his patients knew of his international fame as an and molecular. The clinical age began with Von Hebra’s des- expert on occupational diseases of the skin. He studied at cription of a familial blistering disease in 1870 but it was Edinburgh University and University College London and Koebner who introduced the name epidermolysis bullosa returned to the Wigan coalfield area where he worked as a hereditare 16 years later. Hallopeau made the first attempt to general practitioner for most of his career. He was appointed classify the different clinical types in 1898. He divided EB into Honorary Physician to Wigan Infirmary where he founded the nonscarring disease, EB simplex (EBS); and cases where nail skin department and donated the first X-ray machines and Fin- dystrophy or skin atrophy were prominent, dystrophic EB sen light treatment units. Such was his interest in occupational (DEB). Since Koebner’s first description of a widespread variant dermatoses, he is said to have experimented on his own skin of EBS in 1886, other notable events in EBS include Dowling by wrapping his arm with floured cloths and cheap furs in an and Meara’s description in 1954 of a group of patients with attempt to find cures for the resultant ‘bakers itch’ and fur- features reminiscent of dermatitis herpetiformis and high infant related eruptions. He held several positions of authority during mortality, and the clarification of the Weber-Cockayne variant his career. He was president of the Certifying Factory Surgeons in 1957. The study of DEB produced descriptions of a mutila- Association, Manchester Medical Society and London Dermato- ting form by Hallopeau (1896) and Siemens (1921), and logical Society from 1920–1923 (now the St John’s Hospital reports about dominant DEB by Cockayne (1933) and Touraine Dermatological Society), where he founded an annual oration (1942). In 1935 Herlitz had noted that there was an extreme on the progress of dermatology. The annual lecture became form of EB that lead to death in early infancy (EB letalis) and at known as the Prosser White Oration until 1990 when the that time it was included as part of the dystrophic spectrum. name was changed to the St John’s Oration. He gained inter- The ultrastructural age began with the arrival of electron national recognition and was elected a member of the Perma- microscopic techniques that enabled examination of the skin in nent Committee for the Study of Occupational Diseases, EB patients with a level of detail not possible before. In 1962 reporting to the League of Nations in Geneva. He published Pearson demonstrated distinctive levels of cleavage within EBS many articles and became associate editor of the Journal of and DEB and was also able to show that in EB letalis the split Industrial Hygiene, but his most famous work was his book was not the same as in DEB. Thereafter a third group, junc- The Occupational Affections of the Skin published in 1915 (London: tional EB (JEB), was added to the nomenclature, and in 1976 H.K. Lewis). His text was translated into several languages in- Hashimoto et al. described a nonlethal variant of JEB, later called cluding German and Russian. Despite ongoing problems with non-Herlitz JEB. The molecular age with its advances in immu- cardiac disease he worked until 2 weeks before his death and nological and genetic techniques added to our understanding is quoted as having told his own doctor, who recommended of the pathogenesis of EB. In 1986 Heagerty et al. demonstrated he take some rest, ‘a heart is a muscle and should be kept a lack of staining in DEB with a marker later shown to attach to exercised’. He had a great love for his local community and collagen VII, the main component of anchoring fibrils (Heager- amongst other things he created a trust fund to buy medical ty AH, Kennedy AR, Gunner DB, Eady RA. Rapid prenatal diag- books for the public library, founded a French travelling nosis and exclusion of epidermolysis bullosa using novel scholarship for Wigan students, and commissioned artists to antibody probes. J Invest Dermatol 1986; 86: 603–5). The same paint local buildings, a selection of which works he presented team also found a lack of staining of Herlitz JEB with GB3 anti- to the local library. He had a keen interest in music and left body, subsequently proven to be directed against the anchoring several musical trusts to churches in the region. The inscrip- filament protein laminin 5. In 1991 Lane et al. found a muta- tion of his book The Occupational Affections of the Skin gives some tion in the gene encoding for keratin 5 in Dowling-Meara EBS. insight into the author’s sentiments about literature ‘To read a Discovering abnormalities in these diseases has allowed devel- book is a tool to use, a title to abuse, or a toy to amuse: but opments in diagnosis and prenatal testing for EB and has taught mirrors the soul of the writer’. His memory lives on in the us much about the normal functions of skin. A history of EB, Prosser White Dermatology Centre at the Wigan NHS Trust. however, would not be complete without acknowledging the many individuals and establishments involved in research, epi- demiology, and patient care and support.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 74–80 76 Historical Archive Symposium

H-5 uncle, Astley Cooper in 1821 and studied at St Thomas’s Hos- How skin disease has shaped our history: the pital and Guy’s Hospital, where he was a demonstrator of dermatoses of English Royalty and rulers anatomy. In 1839, he published Practical Anatomy of the Nerves and D. Torley and N.J. Levell Vessels supplying the Head, Neck and Chest known to students as Norfolk and Norwich University Hospital, Norwich, U.K. ‘Cock’s Head and Neck’. He became a full surgeon in 1849 Oliver Cromwell, the Lord Protector of England allegedly and was president of the Royal College of Surgeons in 1869. insisted ‘Paint your picture truly like me…. all these rough- In surgery, his name is connected to a range of important nesses, pimples, warts, and everything’. With these words he work including the pathological changes causing congenital contrasted his utilitarian approach to ruling with many of the deafness, treatment of impermeable urethral stricture, pioneer- vainglorious monarchs that preceded and followed him. With ing pharyngotomy, and he was one of the first surgeons to the stigma associated with skin diseases throughout history it trephine for middle meningeal haemorrhage successfully. In is not surprising that most rulers chose to restrict mention of dermatology, he described the clinical features of four cases of their diseases in literature and portraiture. However skin dis- large proliferating scalp or face tumours which had been diag- eases are common and our research has revealed evidence of nosed as malignant but which he felt were benign (Cock E. A diseases affecting the skin in many English leaders (Table 1). few remarks on a peculiar follicular disease. Guy’s Hospital Reports Such a list involves some speculation and interpretation of 1852–1853; 8: 151–74). The microscopic reports on these contemporary accounts. Henry IV has been considered by pre- tumours were basic but the drawings in his original paper vious authors to have leprosy or pemphigus; however, we suggest the diagnosis of giant proliferative pilar cyst. This consider chronic plaque psoriasis with psoriatic arthropathy to tumour has been the subject of much discussion and debate in be a more likely cause of his disability. This study critically the dermatopathology literature since its initial description and examines the evidence concerning these skin diseases and how there would appear to be a spectrum of both clinical and they may have affected and handicapped the rulers of histological findings possible (Folpe AL, Reisenauer K, Mentzel T England. et al. Proliferating trichilemmal tumours: clinicopathological Table 1. Possible diseases affecting the skin in English Rulers evaluation is a guide to biologic behaviour. J Cutan Pathol 2003; 30: 492–8). The malignant potential of Cock’s peculiar Richard 1 1157–1199 Arrow wound causing cellulitis tumour remains controversial to the present day although Henry IV 1366–1413 Psoriasis with arthropathy Henry VIII 1491–1547 Venous leg ulcer complete surgical excision is recommended. Elizabeth 1 1533–1603 Alopecia (?traction) James 1 1566–1625 Porphyria Oliver Cromwell 1599–1658 Intradermal naevi James II 1633–1701 Syphilis Mary II 1662–1694 Smallpox Anne 1665–1714 Lupus erythematosus George II 1683–1760 Syphilis H-7 George III 1738–1820 Porphyria exacerbated by arsenic The history of hair removal William IV 1765–1837 Syphilis M. Mehmi and A. Abdullah Birmingham Skin Centre, Birmingham, U.K. Humans have removed hair over the ages for religious, aes- thetic and infection control purposes. Hair reduction methods have evolved over time, allowing us to take a fascinating jour- ney through the history of hair removal. Cavemen used sharp- H-6 ened rocks and shells to scrape off hair. Bronze razors have Edward Cock (1805–1892) and his peculiar tumour been recovered from ancient Egyptian tombs. Sumerians and A. Affleck, S. Varma,* P. Graham and C. Guevara Mesopotamians used tweezers. Native Americans tweezed their Department of Dermatology, Ninewells Hospital and Medical School, Dundee, moustaches between halves of a clam shell. Electronic twee- U.K.; *Department of Dermatology, Nottingham University Hospitals NHS zing was developed in the late 1950s. Jean Jacques Perret, a Trust, Nottingham, U.K.; Department of Plastic Surgery, Nottingham Uni- French barber, invented a safety razor for shaving in 1762. versity Hospitals NHS Trust, Nottingham, U.K. and Department of Histo- King Gilette had the idea for a razor with disposable blades in pathology, Nottingham University Hospitals NHS Trust, Nottingham, U.K. 1895. The first electric shaver for men was invented by Jacob A 79-year-old woman presented with a giant, cystic lesion of Schick in 1931. An epilator was first made available in 1986. the scalp. There had been a smaller nodule at the site for 60 Middle Eastern brides had premarital sugaring of unwanted years which had suddenly enlarged over 2 months. A malig- body hair as a symbol of respect for their husbands. A sugar- nant tumour was suspected. Excision biopsy showed histo- based paste made from citrus fruits was pulled off in the logical features of a proliferating pilar cyst or Cock’s peculiar opposite direction of hair growth. Original waxing methods tumour. So who was Cock and are we any wiser as to the nat- used heated wax derived from tallow and resins. Egyptian ure of the entity that he first described in 1852? Edward Cock women waxed their legs using beeswax. Brazilian women was born in Middlesex in 1805. He was apprenticed to his have used Coco de Mono tree secretions as waxing agents.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 74–80 Historical Archive Symposium 77

Modern wax stripping was introduced in the late 1960s. edly sold in Indian pharmacies as chrysatobine. In India it was Threading using cotton string was developed in Arabia. Egyp- applied using a cut lime but in Brazil it was mixed with vin- tians including Cleopatra used threading to maintain a hairless egar. Purified chrysarobin is a mixture of substances (chrysa- face. Early depilatories were derived from pitch, white vine or robin, dichrysarobin and dichrysarobin methyl ether) obtained ivy gum extracts, resin, ass’s fat, bat’s blood, pulverised eggs by extraction with hot chloroform. By 1915 western pharma- shells, cat’s dung and vinegar, quick lime and powdered viper. copoeias listed a number of topical formulations including In 4000 BC a depilatory named rhusma turcorum containing pastes, ointments and tinctures. Pills were made for internal orpiment (natural arsenic trisulphide), quicklime and starch use by incorporating the powder into medicinal soap or gly- was used by women. In the 1700s American women applied cerine of tragacanth but were rarely used because of gastroen- poultices of caustic lye to burn excess hair. Powdered depilat- teritis. Topical application was known to cause ‘a temporary ories were marketed in the USA in 1844. Depilatory agents uneasy sensation’ and staining of the skin but the staining usually dissolve hair keratin by disrupting disulphide bonds was less marked than occurs with dithranol. Shortages of Goa using thioglycates. Sulphuric acid impregnated needles were Powder during World War I prompted Bayer Farbenfabriken twisted into hair follicles in the late 1800s by physicians. This to synthesise 1,8 dihydroxyanthrone (Cignolin, dithranol, process was refined in the 20th century and termed electro- anthralin) as a more reliable substitute which gradually lysis. Long pulsed ruby, alexandrite, diode, Q-switched and replaced chrysarobin, although several pharmacopoeias contin- long pulsed neodymium-doped yttrium-aluminium-garnet ued to list it until at least 1989. It was certainly used success- (Nd:YAG) lasers and intense pulsed light (IPL) were approved fully by some U.K. dermatologists until the early 1980s for for photoepilation in sequential order from the mid-1990s those intolerant of dithranol. onwards (Tanzi E, Lupton J, Alster T. Lasers in dermatology: four decades of progress. J Am Acad Dermatol 2003; 49: 1–34). Hair-inhibitors have been used since the snake-oil heyday of the 19th century. Eflornithine hydrochloride cream inhibits ornithine decarboxylase and was introduced in 2000. H-9 The history of congenital erythropoietic porphyria (Gu¨nther’s disease) R. Katugampola, M. Badminton* and A. Anstey Department of Dermatology, School of Medicine, Cardiff University, Cardiff, H-8 U.K.; *Department of Medical Biochemistry and Immunology, School of A history of Goa powder (chrysarobin): an early Medicine, Cardiff University, Cardiff, U.K. and Department of Dermato- effective treatment for psoriasis logy, Royal Gwent Hospital, Newport, U.K. C. Clark and S. Lewis-Jones Congenital erythropoietic porphyria (CEP, Gu¨nther’s disease) Ninewells Hospital and Medical School, Dundee, U.K. is a rare autosomal recessive disease, caused by a marked defi- Chrysarobin, the precursor of dithranol, and the most effective ciency of the fourth enzyme of the haem biosynthetic path- early therapy for psoriasis was introduced into western medi- way, uroporphyrinogen III synthase (UROS). Its main features cine by an English dermatologist, Balmanno Squire. He first include severe cutaneous photosensitivity to visible light, skin realized its value in the treatment of psoriasis when a patient fragility, scarring and mutilation starting in early infancy, red misdiagnosed psoriasis as ringworm and applied a preparation urine and haemolytic anaemia. To date, only about 130 of Goa powder with great success. In 1878 Squire published a patients with CEP have been reported worldwide. However monograph ‘on the treatment of psoriasis by an ointment of CEP received a lot of attention in the >130-year documented chrysophanic acid’ using a benzene extract of Goa powder. clinical history of human porphyrias, probably due to its stri- Although the first published analysis of Goa powder suggested king clinical features. In 1874 Schultz reported the first case of that chrysophanic acid was the main constituent a later analy- porphyria, a case of CEP then called ‘pemphigus leprosus’: a sis determined that it was chrysarobin. Crude chrysarobin or man with a history of photosensitivity since 3 months old, araroba powder is found in longitudinal cavities in the trunk with blistering and scarring on light exposed skin, anaemia, of the araroba tree (Andira araroba, Leguminosae), growing in splenomegaly and brown-red bones at autopsy. ‘Haema- the forests of Bahia (Brazil). A pale primrose colour when toporphyrin’ (iron-free haematin) identified in the patient’s fresh, it darkens to purple-brown with age. Portuguese mer- red urine was thought to be due to an error in haemoglobin chants brought it from Brazil to Goa where it became known synthesis. Reports of similar cases followed, by Gagey (1896) as Goa powder. Alternative names were Bahia powder, Brazil of two male cousins, McCall Anderson (1898) of two brothers powder and ringworm powder. It had been used in India for (diagnosed as hydroa aestivale), Volmer (1903) of a female, some time as a vermifuge (for Taenia solium, pork tapeworm) Linser (1906) and Grob (1910) a male patient each. McCall and for the treatment of a wide variety of skin conditions Anderson (1898) suggested an association between cutaneous including psoriasis and . Kemp reported the exist- photosensitivity and haematoporphyrin, which Meyer-Betz ence of Goa Powder in India in 1864. In 1874 Fayrer reported (1913) confirmed by self-administering haematoporphyrin its use in the treatment of psoriasis. At that time it was report- and developing marked photosensitivity following sunlight

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 74–80 78 Historical Archive Symposium exposure. In 1911 Hans Gu¨nther reported detailed clinical and began a ‘Pregnancy prevention program for women on accuta- investigative findings of his patient with CEP, Mathias Petry. ne’ after FDA deliberations on withdrawing the license in the Petry worked in the laboratory of Hans Fischer (1930 Nobel USA. Studies showed that fetal exposures, although reduced, Prize winner for work on pyrol chemistry and porphyrins), continued to occur in up to 1 in 250 women treated. In and was the ideal subject for his studies of porphyrins. Fis- response to this, in 2006 a new mandatory management pro- cher’s autopsy examination of Petry confirmed the erythroid gramme, iPLEDGE, was fully implemented in the U.S.A. In nature of CEP. In 1911 Gu¨nther classified all previously de- 2005 the European medicines agency, in collaboration with scribed cases of human porphyrias (haematoporphyrinuria) dermatology organisations including the British Association of into acute, congenital with photosensitivity from birth (CEP) Dermatologists, implemented a far tighter pregnancy preven- and chronic late-onset photosensitivity (porphyria cutanea tion programme for U.K. patients treated by dermatologists. tarda). The essential steps of the haem biosynthetic pathway This should have allowed the safe prescribing of this treatment and biochemical profiles of the different porphyrias were that benefits so many young people. However, this risk man- identified in the 1940s. Photosensitivity to visible light in por- agement programme is not mandatory and has coincided with phyrias was confirmed by Magnus et al. in 1950 (Magnus IA, deregulation for unknown reasons of isotretinoin in the U.K. Porter AD, Rimington C. The action spectrum for skin lesions It is not illegal to purchase isotretinoin from internet pharma- in porphyria cutanea tarda. Lancet 1950; i: 912–14). In 1969 cies and primary care physicians can prescribe isotretinoin UROS deficiency was identified as the cause of human CEP, its (Torley D. Buying isotretinoin on the worldwide web. Br J Der- gene isolated in 1988, gene mutations causing CEP first matol 2005; 153(Suppl.1): 48. Abstract). In February 2007 described in 1990 and the human UROS gene located on chro- the U.K. Medicine and Healthcare Products Regulatory Author- mosome 10q25Æ2-26Æ3 in 1991. The first bone marrow trans- ity verbally informed us that primary care and internet prescri- plantation of a CEP patient resulting in biochemical bing is legal and therefore they can at present do nothing improvement occurred in 1991 (Manchester, U.K.). In-vitro about it. U.K. authorities appear to be ignoring the historical studies commenced in 1995 on fibroblasts and lymphoblasts lessons of the thalidomide tragedy, and responding inad- of CEP patients resulting in significantly high levels of UROS equately to warnings from dermatologists. Have the lessons of expression are promising for future gene therapy as a potential history been forgotten so quickly? cure for CEP.

H-11 H-10 ‘Less Work and Good Beer!’: a historical review of Will the thalidomide tragedy repeat itself in the U.K. fungus and the skin with the increasing availability of isotretinoin? A A.M. Skellett and N.J. Levell history of teratogenic drugs in dermatology Norfolk and Norwich University Hospital NHS Trust, Norwich, U.K. D. Torley and N.J. Levell Superficial mycoses in animals and man have been a cause of Norfolk and Norwich University Hospital, Norwich, U.K. morbidity for centuries. Evidence of fungal skin infections in Despite the thalidomide crisis in 1961-2, clinicians have long Egyptian mummies has been confirmed by DNA analysis. The appreciated the drug’s potential. In 1965 Sheshkin, an Israeli Ebers Papyrus (circa1550 BC) describes the use of henna for dermatologist, made a fortuitous discovery that thalidomide skin diseases; as henna has been noted to have anti-fungal was effective in patients with erythema nodosum leprosum. It properties it may have been treating superficial mycoses. Fun- has subsequently been used in other dermatological diseases. gal infections of the skin were recognized by the ancient Introduced in 1956 as a sedative and morning sickness treat- Greeks and Romans. The Hippocratic writings discuss aphthae, ment, thalidomide tragically caused phocomelia in approxi- which is believed to be oral thrush. According to Celsus (25 mately 10 000 babies (Perri AJ 3rd, Hsu S. A review of BC–50 AD) [Cornelius Celsus. De Medicina (c. AD 14–37)], the thalidomide’s history and current dermatological applications. Romans knew tinea capitus under the name porrigo (Latin ‘to Dermatology Online Journal 2003; 9(3): 5). Recognition of this spread’). Porrigo was later replaced by the term tinea (after was too slow and prompted the introduction of preclinical Tineidae, the clothes moth, whose depredations affected skin animal testing to identify teratogenic compounds before treat- allegedly resembles). The formal association between mycoses ment of humans. Since 1998, thalidomide has been marketed and human skin was first described by Schoenlein in 1839, in the U.S.A. with the STEPS pregnancy program which has who announced the mycotic nature of Trichophyton favus. Much been highly effective in prevented fetal exposure. The Phar- of the initial work into dermatomycology was performed mion program in Europe is based on STEPS and is an exem- from 1841-4 by David Gruby, a Hungarian. He published plary model for safe use of a teratogenic drug. Pre-clinical accounts of T. favus, Microsporum audouini and T. tonsurans. He isol- animal studies suggested isotretinoin was teratogenic. Roche ated the fungus ‘favus’ from infected individuals and grew released the drug in 1982 with warnings to physicians and them on slices of potatoes (at a time before the use of agar). package inserts. This was ineffective, and in 1988 Roche This established the contagious nature of the disease. Mycoses

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 74–80 Historical Archive Symposium 79 of animals have received less attention than those in man and 1788, Dr. Edward Jenner vaccinated his first patient with cow- often have been recognized following the investigation of pox and presented his successful results at the Royal Society. human disease. In 1835 Agostino Bassi, an Italian lawyer and The term vaccination (the word ‘vaccine’ is from the Latin farmer discovered that silkworm muscardine disease was a word for ‘cow’) was born and vaccination became common- mycosis. William Tilbury Fox recorded seven cases of ring- place. In 1967, the World Health Organisation launched a glo- worm in man, contracted from a white horse. In 1908, bal eradication campaign against smallpox. The last natural Adamson recorded that fungal skin disease in calves can cause case of smallpox was recorded in Somalia in 1977. The last ringworm in man leading the Board of Agriculture to issue person to die of smallpox in the U.K. was a photographer at advisory leaflets. Erasmus Wilson, (1809–1884) advised isola- the University of Birmingham Medical School in 1978. ting affected children and giving them a sound animal diet Research on smallpox was being carried out in the same and good British beer (such as ‘Brewer’s porter’!) By 1910, as building at the time. All known stocks of smallpox have been the contagious nature of this infection was better established destroyed but two places in the world have kept cultures of an English ringworm school was set up in Witham, Essex, the virus, the Center for Disease Control in the U.S.A. and the with Tilbury Fox’s brother, Tommy Colcott Fox, as the visit- Institute of Virus Preparations in Russia. Following the after- ing dermatologist. Here, afflicted children could be both trea- math of terrorist attacks in September 2001, concerns regard- ted and instructed. Recommendations at that time included: ing smallpox as a potential bioterrorism agent have been ‘children (with ringworm) should do little if any work and raised. In March 2003, smallpox scabs were found inside an have plenty of out-door exercise’ (Physicians & Surgeons of envelope in a book on Civil War medicine in New Mexico the Principal London Hospitals, The Family Physician. A manual of raising the possibility that smallpox DNA could be extracted Domestic Medicine, 1884. London: Cassell and Co.). With treat- from these and other scabs and variola regenerated. ments like this, one assumes that ringworm was quite a desir- able condition!

H-13 The king of poisons H-12 A. Sergeant and J. Thomson* Smallpox: eradication of a scourge Western Infirmary, Glasgow, U.K. and *Glasgow Royal Infirmary, Glasgow, C. Soon, A. Wong and M. Ogboli U.K. Birmingham Children’s Hospital, Birmingham, U.K. Arsenic (Greek arsenikon, from the Persian Zarnickh, meaning Smallpox was a major killer and one of the most devastating ‘yellow orpiment’) is a naturally occurring element used in diseases known to man. It was first recorded over 3,000 years industry, medicine and felony. As early as 400 BC Hippocrates ago. In the 20th century alone, an estimated three hundred used arsenic to treat ulcers. During the plague people wore million people died from smallpox. Famous sufferers include amulets containing arsenic around their necks to protect from Elizabeth I, Abraham Lincoln and Joseph Stalin. Smallpox is infection. In 1786 Thomas Fowler developed a solution caused by the variola virus. It is a DNA virus. There are two (As2O3), which was used until the mid-20th century to treat main clinical forms of smallpox, variola major and variola scaly skin disorders such as psoriasis, in addition to ague, epi- minor. Variola major is the common and severe form with a lepsy, and rheumatism. Topical preparations were used to treat mortality rate of 30%. Variola minor gives rise to a milder skin cancers, fungal infections, syphilis and scrofula. In 1910 form of the disease with a mortality rate of 1%. The incuba- the Nobel Laureate Paul Elrich developed Salvarsan to treat tion period ranges from 7–17 days. The initial symptoms of syphilis and trypanosomiasis. Victorian women ate arsenic smallpox: fever, fatigue, back pain and headaches last for 2–4 mixed with vinegar and chalk to make their skin paler, or days. Skin lesions progress from erythematous macules to alternatively used it externally to ‘improve complexion’. From papules to pus-filled vesicles. They typically develop in the the middle ages arsenic was used as a homicidal agent, fre- extremities and face before progressing to the trunk. This fea- quently among the ruling classes, earning it the title ‘King of ture helps to distinguish smallpox from . The le- poisons, and poison of Kings’. It is odourless and tasteless and sions eventually form scabs and heal leaving disfiguring, both acute and chronic poisoning result in symptoms that can deeply pitted scars. Smallpox may also cause blindness. Once be confused with a variety of other natural disorders. Symp- symptoms develop, there is no effective treatment and no toms occur within 30 minutes of exposure – the patient known cure. The ancient Indians practised inoculation for experiencing a metallic taste in the mouth and garlicky breath, smallpox. This resulted in a less severe infection. The practice along with xerostomia and dysphagia. This is followed by also spread to China and Africa. This technique is known as severe nausea, vomiting, colicky abdominal pain and profuse variolation. In 1774, Benjamin Jesty successfully inoculated his diarrhoea. Death is usually caused by irreversible circulatory wife and children against smallpox after noticing that dairy- insufficiency, but if the dose is not large enough to kill the maids who caught cowpox did not develop smallpox. In patient a number of secondary effects can be seen 2–4 weeks

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 74–80 80 Historical Archive Symposium after ingestion. These include alopecia, Mee’s lines on the ive treatment was available till the mid-20th century and nails, peripheral neuropathy, renal failure and skin changes patients were considered untouchables. Spinalonga, an island such as those seen in chronic poisoning. Chronic poisoning is off the coast of Crete, was one such leper colony that existed much more insidious – the earliest cutaneous manifestation is as recently as the 1950s. Leprosy sufferers on this island occu- persistent erythematous flushing. Hyperkeratosis then occurs pied an ambiguous status between that of a patient and a pris- with desquamation of the palms and soles, brittle nails with oner. It became synonymous with human misery, as infected Mee’s lines, patchy or diffuse alopecia, facial or ankle oedema, individuals were virtually imprisoned within the shores of this and pigmentary changes. Arsenical keratoses are the most island. In spite of the isolation of patients with leprosy, no characteristic skin feature – they usually present as small, change in the incidence of leprosy was noted. It was only punctuate, symmetrical papules on the palms and soles. with the advent of dapsone that leprosy was finally controlled Hutchinson first reported skin cancer resulting from arsenic and nearly eradicated in Europe. Many governments including therapy in 1888. Bowen’s disease, squamous and basal cell the United States, South Africa, Japan and Colonial India fol- carcinomas may have a long latent period between exposure lowed this isolation policy for leprosy patients. Mycobacterium and development. Other features of chronic intoxication leprae, the causative organism of leprosy, was discovered by include anaemia, irritability, GI upset, neuropathy, encephal- Armauer Hansen in 1873. Even though the exact method of opathy and internal malignancy. Today, it is estimated that transmission is unknown, close contact with the infected indi- approximately 57 million people worldwide are exposed to vidual may be required. It could be spread by aerosol spread arsenic-tainted drinking water. As2O3 (Trisenox) was licensed of nasal secretions and uptake through nasal or respiratory by the FDA in 2000 for use in acute promyelocytic leukaemia, mucosa. The organism has a very long replication period and and arsenic may still be found in some Chinese herbal reme- this may account for the protracted incubation period for this dies. Thus in the 3rd millennium of its use, the dermatologist condition. Leprosy is asymptomatic for months or years, and must remain vigilant for the stigmata of the ‘King of Poisons’. the deformities associated with advanced disease occur in only a few patients. Infected individuals could therefore transmit the condition for years without their own knowledge. Domi- ciliary treatment for tuberculosis has revolutionized the manage- ment of M. tuberculosis and has been shown to be superior to H-14 the use of sanatoria for patients with tuberculosis. Similarly, Spinalonga: the island of lepers treatment of leprosy in the community with potent antimyco- P.D. Yesudian bacterial drugs can cure the disease without the need for isola- Countess of Chester Hospital, Chester, U.K. tion of infected patients. Use of this knowledge could have Leprosy or ‘Hansen’s disease’ is a chronic infectious disorder prevented countless tales of separation and anguish for patients that has long been stigmatised. From Biblical times, an isolation and avoided the dreaded stigma of being branded a leper in policy to segregate patients from society has been used to con- an isolated island. This mark of disgrace, physically present in tain and manage this problem. This policy caused deprivation the sufferers’ disfigured bodies and embodied in the identity of civil liberties and further fostered social stigmas. No effect- of a leper has cast leprosy into the shadows of society.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 74–80 British Contact Dermatitis Society: Summaries of Papers

CD-1 order to identify new allergens. The current literature would Contact dermatitis to octocrylene suggest that octocrylene should be incorporated into the regu- G. Kaushal and D. Orton lar sunscreen series. It has been commercially available from Amersham Hospital, Amersham, U.K. Chemotechnique (Vellinge, Sweden) since January 2005. Currently the most common U.K. photoallergens appear to be sunscreen chemicals (Scheuer E, Warshaw E. Sunscreen allergy: a review of epidemiology, clinical characteristics, and responsible allergens. Dermatitis 2006; 17: 3–11). Octocrylene (2-ethylhexyl-2-cyano-3,3-diphenylacrylate) is a UVB chem- CD-2 ical photoprotectant, that has been used in sunscreens for less Facial and eyelid eczema caused by allergic than a decade. It has been previously described as a nonaller- reactions to colour pigments in foundation genic, nonirritant molecule showing no mutagenic effect. makeup in nickel-sensitive patients These properties have contributed to its widespread use in I. Foulds sunscreens and cosmetic products. We describe two patients The Birmingham Skin Centre, Birmingham, U.K. that have presented in the last year who are allergic to octo- We report five cases of facial eczema. All patients were female crylene. A 37-year-old lady gave a history of developing an and all reacted to one of their foundation products on patch erythematous blistering reaction 1 h after sun exposure. She testing. All the patients were nickel positive on patch testing was also using a sunscreen – Ambre Solaire Kids 30 Colour with a history of previous nickel intolerance (costume jewel- Control – but described getting the reaction when not using lery reaction). Testing with the individual ingredients of their sunscreens. Photoprovocation tests confirmed an underlying foundation products produced negative reactions at 48 and 96 diagnosis of polymorphic light eruption, and patch testing h. All the products from different manufacturers contained revealed allergies to lanolin alcohols (30% pet), Myroxylon perei- four common colour pigments: CI 77492, CI 77489, CI rae (25% pet), Amerchol L 101 (50% pet) and octocrylene 77499 and CI 77491. Patch testing with the individual colour (10% pet). Positive reactions also occurred with Ambre Solaire pigments produced negative reactions at 48 and 96 h. Each of Kids 30 Colour Control and Vichy 60 applied ‘as is’. Irradiated these colour pigments are iron oxide pigments and are known sunscreen chemicals revealed no photoaugmentation reactions. to contain trace amounts of nickel salts, below the level expec- An 8-year-old boy presented with an eczematous rash over ted to elicit an allergic reaction. However, we believe that the the sites of application after using Ambre Solaire Kids SPF 25 addition of three or more of these pigments in a product is Moisturising High Protection Milk . Patch tests and photo- sufficient, when combined with a penetration enhancer such patch tests revealed positive reactions to Ambre Solaire Kids as propylene glycol or butylene glycol, to elicit an allergic SPF 25 Moisturising High Protection Milk and Vichy 60 reaction in an individual known to be allergic to nickel. There applied ‘as is’. Subsequent patch testing revealed contact are at least 10 different iron oxide pigments potentially used allergy to octocrylene (10% pet). Six cases of allergic reactions in foundation products but so far we have only found reac- to octocrylene have been described in the literature within the tions to products containing the combination of CI 77492, CI last 4 years. The first report in 2003 describes two patients 77489, CI 77499 and CI 77491 pigments. We believe that CI with photoallergic contact dermatitis to octocrylene (concen- 77492, CI 77489, CI 77499 and CI 77491 pigments should tration and vehicle not specified). The second article also not be used in combination in foundation makeup products reported two children who developed an allergy to octocry- but that they could be used individually. An analysis of iron lene, and the first case of allergic contact dermatitis to the oxide pigments and cosmetic products will be presented. For sunscreen chemical. The multicentre photopatch study group the last year we have been advising patients with facial eczema (Bryden AM, Moseley H, Ibbotson SH et al. Photopatch testing who are nickel sensitive to avoid products containing CI of 1155 patients: results of the U.K. multicentre photopatch 77492, CI 77489, CI 77499 and CI 77491 and none of these study group. Br J Dermatol 2006; 155: 737–47) recently repor- patients have required any follow-up for ongoing facial ted that the pattern of photoallergic contact dermatitis to sun- eczema. We believe that we have discovered an important hid- screen chemicals was evolving. Octocrylene was not included den source of nickel in foundation makeup products, which in the photoallergen series, but was present in the composi- may be causing unexplained allergic reactions in nickel-sensi- tion of commercial sunscreen products. The above cases illus- tive patients. trate the need to continually modify patch-test batteries in

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 81 82 British Contact Dermatitis Society

CD-3 CD-4 Positive rates to propylgallate on patch testing: a Cheilitis: patch testing, more than just lip service? change in trend J. Ingram, T. Hughes and N. Stone A. Perez, D. Basketter,* I.R. White and Department of Occupational Dermatology, St Woolos Hospital, Newport, J.P. McFadden Gwent, U.K. St John’s Institute of Dermatology, London, U.K. and *S.E.A.C. Unilever, A study of patients presenting with cheilitis was undertaken to Bedfordshire, U.K. determine what proportion of cases was due to allergic contact Propylgallate (E310) has, until recently, been used as a major dermatitis (ACD) and to identify common relevant allergens. antioxidant in fatty food and, in the cosmetic industry, in the This was achieved by a retrospective analysis of the 1744 manufacture of lipsticks. Propylgallate has a high sensitizing patients referred to our contact dermatitis clinic over a 3-year potential; however, the frequency of allergic contact dermatitis period from 2004 to 2006. Forty-four cases of cheilitis were from antioxidants of the gallate type was previously thought identified (3% of the total) of whom the majority were female to be surprisingly low. Previous exposure and orally induced (77%). Nineteen patients had at least one positive patch test tolerance, as suggested by Khan and colleagues, may have result and in 16 (36% of cheilitis patients) this was a relevant explained the low rates of allergic contact dermatitis to pro- finding. Irritant contact cheilitis occurred in 13 patients, often pylgallate in the past (Kahn G, Phanuphak P, Claman HN et al. due to ‘lip licking’. Atopic eczema was diagnosed in 10 Propyl gallate-contact sensitization and orally-induced toler- patients. Alternative diagnoses in the remaining six patients ance. Arch Dermatol 1974; 109: 506–9). In 1992, Hausen and included contact urticaria (four patients), oral allergy syn- Beyer reviewed the literature and frequency of allergic contact drome (one patient) and angio-oedema (one patient). The 16 sensitization to gallates and found it to be increasing, propyl- patients with cheilitis due to ACD had 44 relevant positive gallate being the most commonly implicated of the gallates patch-test results. Forty-five per cent of these were on the (Hausen BM, Beyer W. The sensitizing capacity of the antioxi- standard battery and most of the remainder (39%) were on dants propyl, octyl and dodecyl gallate and some related gallic the oral battery. Fragrance and flavour additives in lipstick, lip acid esters. Contact Dermatitis 1992; 26: 253–8). A recent report salve and toothpaste were the most common allergens, (2005) by the Cosmetic Review Expert Panel published in the accounting for 20 of the positive tests. Examples included U.S.A. states that in spite of an increased use of propylgallate vanilla in a lip salve, limonene in toothpaste and Myroxylon in the cosmetic industry (although at lower concentrations pereirae in both lipstick and Earl Grey tea. Preservatives such as that in previous decades), sensitization rates have not benzoic acid, propylgallate and sodium metabisulphite were increased (Bergfeld WF, Belsito DV, Klaassen CD et al. Amen- the next most common group, responsible for seven positive ded final report of the Cosmetic Ingredient Review Expert tests. Other more diverse allergens included para-phenylenedi- Panel. Safety assessment of propylgallate. 13 September 2005). The amine in a man who dyed his moustache, and acrylates in a aim of this study was to assess the prevalence of allergic con- woman with false nails. ACD was the most common diagnosis tact dermatitis to propylgallate in our centre from 1988 to in patients presenting to our clinic with cheilitis. This con- 2005. From 1988 to 2005, 9529 patients were patch tested to firms the results of previous published series which suggest the face series, 6973 were women and 2556 were men. Patch ACD as the cause of approximately one-third of cheilitis cases tests were read at 48 and 96 h. Positive reactions were scored (Freeman S, Stephens R. Cheilitis: analysis of 75 cases referred according to the International Contact Dermatitis Research to a contact dermatitis clinic. Am J Contact Dermat 1999; 10: Group recommendations as negative, +, ++ and +++ reac- 198–200). Patch testing with both the standard and oral bat- tions. Propylgallate was used at a 1% pet. A total of 55 teries is an important investigation in patients with this pre- patients had positive reactions to propylgallate 1% pet sentation. (0Æ57%), 46 were female (0Æ65%) and nine were male (0Æ33%). Using v2 test, there was a significant difference (P <0Æ05) in the positivity rates between the 1988–1997 period (0Æ45%) and the 1998–2005 period (0Æ77%). A review of our face series performed in the last 18 years has shown a CD-5 statistically significant increase in propylgallate positive rates Allergic contact dermatitis to hydroxydecyl on patch testing over the past decade. An increase in its use in ubiquinone: a newly described contact allergen the cosmetic industry may well be the explanation for this. in cosmetics Nevertheless, a concomitant reduction of propylgallate as an J.D. Fleming, J.M.L. White and I.R. White antioxidant in food, with oral tolerance being less likely to St John’s Institute of Dermatology, London, U.K. develop, may also be a contributing factor in the increasing A 50-year-old woman, an editor on the beauty section of a trend of allergic contact dermatitis caused by propylgallate. magazine, was referred by her GP following a facial rash that developed after using Prevage MD, an antioxidant facial cream marketed by Elizabeth Arden, for the first time. Approximately 4 h following its application, her face felt hot and tight and she noticed facial reddening. By the next morning,

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 British Contact Dermatitis Society 83 her face and upper chest were red and swollen and she had composition varies depending on the source (e.g. poplar tree developed periorbital oedema. These areas remained itchy for in central Europe). Propolis is widely used in ‘natural’ cosmet- 2 weeks; the rash settled with dryness and scaling. She had no ics, remedies and over-the-counter products. Allergy to propo- history of contact allergy and was not atopic. Patch testing lis is common and cross-reaction with fragrance mix I, was performed to an extended European standard series, face colophonium and Myroxylon pereirae can occur (Wohrl S, Hem- series, skin care allergens and to the diluted ingredients of mer W, Focke M et al. The significance of fragrance mix, bal- Prevage MD provided by the company. The only positive reac- sam of Peru, colophony and propolis as screening tools in the tion at either 2 or 4 days after application was to hydroxyde- detection of fragrance allergy. Br J Dermatol 2001; 145: 268– cyl ubiquinone (graded ++ on both readings). Hydroxydecyl 73). Because of its common origin, propolis could occur in ubiquinone (C19H30O5), also known as idebenone, is an anti- beeswax, a common excipient in topical applications. oxidant and the active ingredient used in Prevage MD. It is a Although propolis sensitivity was reported to be 1Æ2–4Æ2% compound of the quinine family and is promoted commer- (Wohrl et al.), a higher frequency of allergy to propolis (9%) cially as a synthetic analogue of coenzyme Q-10. It has has been reported recently (Giusti F, Miglietta R, Seidenari S. become popular in ‘antiageing’ topical products. The hydroxy- High frequency of sensitization to propolis in a patch test decyl ubiquinone had been diluted in petrolatum to a concen- population of 1255 children. Pediatr Dermatol 2004; 21: 355 tration of 0Æ5% for patch testing, the same level as present in Abstract). Propolis allergy is increasing and it is going to be the cosmetic product. This compound was also tested at the included in the British Contact Dermatitis Society (BCDS) same concentration in 20 controls with no reaction noted in standard series. We investigated the rate of cross-sensitivity any subject at 2 or 4 days, indicating that the positive reaction between propolis, balsam of Peru, colophony and fragrance in our case was unlikely to be an irritant effect. The history of mix, and whether patients with allergy to propolis are allergic desquamation as the reaction settled indicated that this was a to beeswax. In the BCDS patch-test database of 10 826 dermatitic, not urticarial, response and that the likely cause patients, 53 out of 2185 subjects who were patch tested with was an allergic contact dermatitis to hydroxydecyl ubiquinone. propolis in nine U.K. centres were found to have positive The subject must have been exposed previously to this sub- results. Out of 53 positive results 13 were of current relevance stance in another cosmetic product for her to have acquired and three were of past relevance, whereas relevance was not contact allergy to it. This is the first documented case of aller- known in 14 and was not reported in nine subjects. Out of gic contact dermatitis to hydroxydecyl ubiquinone that we are these 53 subjects, four (8%) subjects were allergic to beeswax, aware of. We suspect that such reactions may be more com- 21 (40%) to Myroxylon pereirae, 15 (28%) to colophonium and mon and propose that it be tested for more widely in suspec- five (9%) to fragrance mix I. We sent a survey questionnaire ted cosmetic allergic contact dermatitis. to all nine centres and received additional data from records of 41 subjects at seven centres. Hands were the most common sites of involvement in 23 subjects followed by the face in nine. Eczema involved the lower limbs in seven and was gen- eralized in five subjects. Cosmetics were the most common CD-6 source of contact in 21 and the source was not known in 10. The importance of propolis in patch testing: a Propolis allergy was thought to be occupational in eight. Self- multicentre survey medication and Sudocream were sources of propolis in two S. Rajpara, A.D. Ormerod, M.S. Wilkinson,* C.M. King, and one subjects, respectively. Out of these 41 subjects 12 D.J. Gawkrodger, J.S.C. English,§ B.N. Statham,– subjects were followed up in the clinic and eight reported C. Green,** J.E. Sansom and M.M.U. Chowdhury improvement in eczema following avoidance of the substances Dermatology Department, Aberdeen Royal Infirmary, NHS Grampian, containing propolis (six reported marked and two reported Aberdeen, U.K.; *Dermatology Department, Leeds General Infirmary, Leeds, moderate benefits). In conclusion, propolis is an important U.K.; Dermatology Department, Royal Liverpool and Broadgreen Hospitals, allergen of increasing frequency and its inclusion in BCDS Liverpool, U.K.; Dermatology Department, Royal Hallamshire Hospital Uni- standard series is appropriate. Cross-sensitivity to beeswax is versity of Sheffield, Sheffield, U.K.; §Dermatology Department, Queen’s Med- rare. ical Centre, Nottingham, U.K.; –Dermatology Department, Singleton Hospital, Swansea, U.K.; **Dermatology Department, Ninewells Hospital, Dundee, U.K.; Dermatology Department, Bristol Royal Infirmary, Bristol, U.K. and The Welsh Institute of Dermatology, Cardiff, U.K. Propolis or ‘bee glue’ is a generic name for resinous sub- stances, collected by honeybees from various trees, whose

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 84 British Contact Dermatitis Society

CD-7 a Danish schoolgirl population with ears pierced after imple- The frequency of nickel allergy in patch-tested mentation of a nickel-exposure regulation. Br J Dermatol 2002; female patients: age-stratified data from nine 146: 636–42). Our data differ because they are from a patch- U.K. centres for 2004–2005 tested population, but even so they suggest that the frequency C.L. Thomas, B.N. Statham, M.M.U. Chowdhury,* J.S.C. English, D.J. Gawkrodger, C.M. Green,§ of positive patch-test reactions to nickel peaks at 35–39 years C.M. King,– A.D. Ormerod,** J.E. Sansom and of age in females. We have provided data stratified into bands M.S. Wilkinson of 5 years for the younger age groups (from 10 to 49 years) Department of Dermatology, Singleton Hospital, Swansea, U.K.; *Department which would allow for comparisons to be made with future of Dermatology, University Hospital of Wales, Cardiff, U.K.; Department studies. It will be worth repeating this analysis on the com- of Dermatology, Queen’s Medical Centre, Nottingham, U.K.; Department of bined data for 2009–2010 from these nine U.K. centres. Dermatology, Royal Hallamshire Hospital, Sheffield, U.K.; §Department of Dermatology, Ninewells Hospital, Dundee, U.K.; –Department of Dermatol- ogy, Royal Liverpool University Hospital, Liverpool, U.K.; **Department of Dermatology, Aberdeen Royal Infirmary, Aberdeen, U.K.; Department of Dermatology, Bristol Royal Infirmary, Bristol, U.K. and Department of CD-8 Dermatology, Leeds General Infirmary, Leeds, U.K. A survey of contact dermatitis services in the U.K. Since July 2001 European Union (EU) regulations have pro- A. Mehta and B.N. Statham hibited the sale of any new product that is intended to come Singleton Hospital, Swansea, U.K. into direct and prolonged contact with the skin which releases Guidelines to management of contact dermatitis were pub- more than 0Æ5 lgcm–2 of nickel per week. The female popu- lished in the British Journal of Dermatology in 2001 (Bourke lation is particularly at risk of nickel sensitization mainly J, Coulson I, English J. Guidelines for care of contact derma- because of their very high rates of ear-lobe piercing and of titis. Br J Dermatol 2001; 145: 877– 85). The aim of our study wearing nickel-releasing jewellery. It is yet to be seen to what was to gauge the range of current practice in U.K. dermatol- extent the new EU regulations will reduce the prevalence of ogy departments. We sent 157 questionnaires to lead clini- nickel allergy in the U.K. Since January 2004 nine U.K. centres cians in every U.K. dermatology department; to date, 60 have been regularly contributing patch-test data to a central replies have been received. The questionnaire consists of 26 database. We investigated the frequency of nickel allergy questions relating to practice based on the earlier guidelines. amongst sequential patients who had undergone patch testing Ninety per cent of dermatologists performed patch testing in to the British Contact Dermatitis Society (BCDS) standard bat- their own departments. Fifty-eight per cent of dermatologists tery from 1 January 2004 to 31 December 2005 (total num- performed patch-test readings themselves or jointly with nur- ber tested = 6857). Overall, the positive patch-test rate to ses, specialist registrars or NCCG doctors. Nurses performed nickel (irritant reactions excluded) was 1279 of 4562 (28%) patch-test readings in nine centres of which eight were super- of females tested, and 183 of 2295 (8%) of males tested. The vised by doctors. Eighty-two per cent of centres performed frequency of positive patch tests to nickel in females was: 1 of readings on days 2 and 4; day 7 readings were performed by 58 tested (2%) at age 0–9 years; 19 of 135 (14%) at 10–14 37%. Eighty-seven per cent of centres used the British Contact years; 70 of 320 (22%) at 15–19 years; 131 of 418 (31%) at Dermatitis Society (BCDS) standard battery or the BCDS stand- 20–24 years; 120 of 373 (32%) at 25–29 years; 141 of 361 ard with additional allergens for routine screening. Other bat- (39%) at 30–34 years; 157 of 394 (40%) at 35–39 years; teries used by dermatologists include cosmetic, corticosteroid, 154 of 437 (35%) at 40–44 years; 126 of 373 (34%) at 45– hair and medicament, which were tested by more than 78%. 49 years; 188 of 752 (25%) at 50–59 years; 101 of 480 Plants, oils, acrylates and fragrances were tested by a small (21%) at 60–69 years; 57 of 343 (17%) at 70–79 years; 14 number of centres only. The number of patients tested per of 118 (12%) for ages > 80 years. In males, there was a posi- week varied widely between < 5 to > 30, with 32% testing tive nickel patch-test rate of 8–10% between the ages of 20 1–5, 36% 6–10, 22% 11–20, 5% 21–30 and 3% testing more and 60 years, without an obvious peak. For comparative pur- than 30 patients per week. Allergens were stored in the fridge poses, the frequency of positive patch tests to fragrance mix I by 98% of centres. A database was used to record results by and Lyral were recorded for the same age groups over this 63%, with 15 centres using the BCDS database and 23 their time period. In females, positive reactions were seen to these own database; 35% did not record results on any database. fragrances in between 3% and 12% of those tested, with the Allergen information leaflets are given by 97% of dermatolo- peaks being between ages 45 and 69 years. In Denmark a sta- gists. SPR training is performed in 28 dermatology centres; 19 tutory order regulating nickel-containing products was imple- SPRs were supervised on all occasions, six regularly and three mented in 1992. One Danish group studied girls aged 10–14 infrequently. Contact dermatitis textbooks are available in 92% and girls aged 17–22 in school populations in 1999–2000, of dermatology departments and one or more contact derma- and suggested that there were already some signs that the titis journals in 48%. Photopatch testing was performed by nickel regulations were having a beneficial effect (Jensen CS, 52% of dermatologists, latex skin prick testing by 58%, and Lisby S, Baadsgaard O et al. Decrease in nickel sensitization in IgE RAST to latex by 97%. In pregnancy, 18% of centres skin

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 British Contact Dermatitis Society 85 prick test and 28% patch test. This survey confirms high CD-10 standards of compliance with existing guidelines in a majority Home patch-test kits for allergy to para- of centres and will guide the setting of core standards for the phenylenediamine: do they help the consumer? practice of contact dermatitis in U.K. departments. D. Orton Department of Dermatology, Amersham Hospital, Buckinghamshire, U.K. In 2004, a U.K.-based company developed and marketed a patch-test kit (Colourstart, Trichocare Diagnostics, Bedford. U.K.), to hairdressers designed to assess whether their clients CD-9 had a ‘problem with hair dye’ and promoting itself as the T.R.U.E. testÒ as a reliable method for patch testing ‘only practical solution to sensitivity testing’. The test con- in the community? sists of a temporary transfer containing para-phenylenediam- I. Foulds ine (PPD) and resorcinol, both at nondocumented The Birmingham Skin Centre, Birmingham, U.K. concentrations. Clients are advised to follow the simple With the current emphasis on shifting dermatology services guidelines for use and apply the test 48 h prior to having into the community the role of patch testing in the com- their hair coloured at a salon. The hairdresser or beauty munity is also being promoted. One such test is the therapist is then advised to remove the transfer in the salon T.R.U.E. test (Allerderm, Phoenix, AZ, U.S.A.) which is and assess for unusual sensations, irritation, redness or being advertised as an easily performed test that can be swelling. If these are present, it is recommended that the undertaken with little training giving reliable results. General client is advised that they are ‘hypersensitive’ and should practitioners and nurses with no specific training are being not have their hair coloured. The company website main- encouraged to adopt this investigation in the community. tains that Colourstart delivers a safe and measured amount The literature promoted by T.R.U.E. test states that a nega- of PPD to the surface of the skin and ‘accurate dosing’. To tive result is very common in six out of 10 patients tested. date, we have been unable to obtain technical details of this They also state that ‘Your negative result will help your product or the results of clinical trials. The product first doctor treat your skin condition correctly so that it can came to light when a patient was referred to the depart- begin to heal’. If reliance in the future is going to be based ment having experienced an adverse reaction to a hair col- upon T.R.U.E. testing in the community, how many cases ouring product that was not detected by the salon using of allergic contact dermatitis will be missed by lack of full the Colourstart kit prior to applying the colour. Conven- investigation in a specialized centre? We have undertaken tional patch testing in this patient using PPD (1% pet) was an analysis of the frequency of positive results that we positive along with toluene-2,5-diamine sulphate (1% pet) might have obtained by using the 24 standardized T.R.U.E. (PTD). Subsequently, we retrospectively assessed the Colour- test allergens compared to the number of reactions seen by start kit in the next six patients presenting to our clinic more extensive investigation using other allergens and with a history of adverse reactions to hair colours, and patients’ own products and work materials. One hundred demonstrating allergic reactions to the main hair dye chemi- consecutive cases attending the patch-test clinic were ana- cals on conventional patch tests. Of these patients, one lysed for frequency of reactions to the 24 T.R.U.E. test patient allergic to PTD alone was not detected with the kit, allergens compared to reactions that would have been and three patients reacting to PPD alone on conventional missed by not testing to other potential allergens. A full an- patch tests were not detected with the kit. The two patients alysis of the data will be presented. However, it was found who were detected by the kit had positive allergic reactions that by testing to the T.R.U.E. test alone, 60% of other po- to both PPD and PTD on conventional tests. These cases tentially relevant allergens would have been missed. The fact will be discussed in more detail with pictorial evidence. that the T.R.U.E. test is being promoted as a reliable way The results raise concerns about the use of this particular of diagnosing allergic contact dermatitis in the community kit, but also raise several important issues including the may hold true for the limited allergens tested but will regulation and use of ‘home’ patch-test kits, the interpret- grossly under diagnose cases of allergic contact dermatitis. ation of tests by untrained individuals and the ‘one size fit This can only be a disservice to patients and will contribute all’ approach to allergy testing. It is clear that adverse reac- to unnecessary morbidity, the need for ongoing follow-up tions and, in particular, type IV hypersensitivity reactions to and treatment and will ultimately be a more costly ap- hair colouring products, might result from one ingredient proach to treating patients in the community. or a combination of different ingredients. Further discussion on the different allergens that exist within hair dye products will also be presented. Consumer testing of hair dye prod- ucts prior to use is still recommended, but the available tests are still far from perfect.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 86 British Contact Dermatitis Society

CD-11 CD-12 Cross-reactivity of isothiazolinone and Assessment of early outcomes in occupational formaldehyde-releasing preservatives contact dermatitis B.N. Statham, C.M. Green,* C.M. King, J. Williams, A. Lee* and R. Nixon* A.D. Ormerod, J.E. Sansom,§ J.S.C. English,– The Dermatology Centre, Hope Hospital, Manchester, U.K. and *Occupational M.S. Wilkinson,** D.J. Gawkrodger and Dermatology Research and Education Centre, Skin and Cancer Foundation, M.M.U. Chowdhury Melbourne, Australia Swansea NHS Trust, Swansea, U.K.; *Ninewells Hospital, Dundee, U.K.; Contact dermatitis is the most common form of occupational Royal Liverpool Hospital, Liverpool, U.K.; Aberdeen Royal Infirmary, skin disease (OSD), accounting for 79–95% of all cases of Aberdeen, U.K.; §Bristol Royal Infirmary, Bristol, U.K.; –Queen’s Medical OSD. The annual incidence of occupational contact dermatitis Centre, Nottingham, U.K.; **Leeds General Infirmary, Leeds, U.K.; Royal (OCD) has been estimated at 50–190 per 100 000 workers Hallamshire Hospital, Sheffield, U.K. and University Hospital of Wales, and it has been highlighted as an area of significant public Cardiff, U.K. health importance because of its prevalence and economic We set out to investigate the cross-reactivity of biocide pre- impact. There is significant evidence that OCD can have a poor servatives contained within the British Contact Dermatitis Soci- outcome. This is best exemplified by persistent postoccupational ety (BCDS) standard series. It is well recognized that dermatitis: an ongoing dermatitis for which there is no obvi- formaldehyde-releasing biocides show frequent cross-reactions ous present cause, precipitated by prior OCD. Previous studies with each other and formaldehyde, based to a large extent on have focused on the investigation of potential poor prognostic the quantity of formaldehyde released (Jacobs M, White I, factors, comparing outcomes in workers with different diagno Rycroft R, Taub N. Patch testing with preservatives at St John’s ses, workers with atopic diathesis, and the presence of specific from 1982 to 1993. Contact Dermatitis 1995; 33: 247–54). The allergies. We have recently developed an ‘occupational contact likelihood that preservatives not releasing formaldehyde would dermatitis outcome algorithm’ (OCDOA). This describes 22 show any cross-reactivity appears not to have been previously reasons why workers may improve, or fail to improve after investigated. Nine centres using the BCDS database participated being diagnosed with OCD. These OCDOA outcomes are in a data-sharing exercise agreeing to pool anonymized data directly linked to actions and recommendations (investigation, including patient demographics and results of patch-testing education and treatment), aimed at improving the prognosis. results for the BCDS standard series. In total, 6948 patients When used in a long-term follow-up study of OCD, it was were tested to the BCDS standard series. Nine centres contri- apparent that important factors were failure to attend for sub- buted data for the years 2004/05, the preservative allergens sequent follow-up, and poor patient recall of the diagnosis and tested over this period remain unchanged (formaldehyde 1% advice. The aim of this study was to use the OCDOA in the fol- aq, parabens 16% pet, quaternium-15 1% pet, Me/Cl isothiaz- low-up assessment of patients diagnosed with OCD in a ter- olinone 0Æ01% aq, bromo-nitropropane-diol 0Æ25% pet, imid- tiary referral clinic, 3–6 months after initial diagnosis. Over an azolidinyl urea 2Æ0% pet, diazolidinyl urea 2Æ0% pet, methyl- 8-month period, in excess of 50 patients were assessed and dibromo-glutaronitrile 0Æ3% pet, chloroxylenol 0Æ5% pet). In diagnosed with OCD in the clinic, and subsequently reviewed. total, 139 patients tested positive to formaldehyde (2Æ0% of The most common reason for improvement at time of review total tested); the simultaneous reactions with formaldehyde was behavioural modification, followed by job modification. are as follows: quaternium-15 73/139 (54%), imidazolidinyl The most common reason for a failure to improve was the urea 33/139 (24%); diazolidinyl urea 42/139 (30%); worker choosing not to, or being unable to follow the advice bromo-nitropropane-diol 14/139 (10%); Me/Cl isothiazoli- given in the clinic. Patient recall and understanding of the none 22/139 (16%); methyl-dibromo-glutaronitrile 4/139 diagnosis at time of review was often poor. A skin school for (3%); parabens 3/139 (2%); chloroxylenol 1/139 (1%). A workers diagnosed with OCD is due to be implemented in similar pattern of reactivity was evident with quaternium-15- 2007 and should hopefully improve retention of important positive patients (2% of total tested), 9% reacting to bromo- advice. In summary, outcomes in OCD are multifactorial. nitropropane-diol and 18% to Me/Cl isothiazolinone. It is Whilst original diagnosis, presence of atopic diathesis and spe- apparent that the number of simultaneous positive reactions to cific allergies may be important, so too are individual factors Me/Cl isothiazolinone and to formaldehyde or to quaternium- such as diagnostic complexity, patient understanding and 15 is greater than the number of reactions to bromo-nitropro- patient attendance at follow-up. Better outcomes are seen in pane-diol (a formaldehyde-releasing biocide) and substantially cases where workers may easily modify their behaviour to greater than the simultaneous reactions to parabens, chloroxy- avoid the relevant irritant or allergen. Conversely, a poor out- lenol and methyl-dibromo-glutaronitrile (nonformaldehyde come is seen in workers who are unable to, or choose not to releasers). The reason for this association is unclear; there is follow advice. An example of the latter is the healthcare no apparent structural similarity, nor can the association be worker with hand dermatitis unable to avoid irritants in the based on linkage of use in similar product types, as other non- workplace: hand washing and glove wearing. formaldehyde biocides have an identical spectrum of product distribution.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 British Contact Dermatitis Society 87

CD-13 following days the swelling persisted and was associated with Study of morphological patterns, allergens and the development of erythema and vesicles. Her occupation relationship to atopy in patients with hand eczema involved the regular use of polyurethane and epoxy resins, S. Handa, T. Gupta and I. Kaur aerosol paints and fibreglass. She reported the routine use of Postgraduate Institute of Medical Education & Research, Chandigarh, India full recommended barrier equipment and denied any change Hand eczema is a very distressing condition that affects in her usual practice or contact with different substances prior 11–30% of the general population in various countries. It to the acute event. On examination, there was dramatic facial gives rise to a lot of physical and emotional morbidity and oedema with overlying crusted papulovesicles. Bilateral clus- involves several important issues such as worker compensation tered papular lesions also affected the dorsal aspects of her and disability. This study is an attempt to study morphological hands, wrists and fingers. The rest of her skin was unaffected patterns, allergens and relationship to atopy in patients with and respiratory symptoms and signs were absent. Patch testing hand eczema attending the contact dermatitis clinic at a ter- to the British standard and cosmetic/preservative series tiary care referral centre in India. One hundred consecutive revealed a strongly positive reaction to para-phenylenediamine patients with hand eczema were studied. The male to female and a positive reaction to glyceryl monothioglycolate. She did ratio was almost 2 : 1. Mean age of the patients was 38.9 remember a previous reaction to hair dye but denied recent years (range 10–70). Masonry was the main occupation in exposure to hair dyes or perming solution. Patch testing to 33% of the men, while housewives comprised 82% of the the isocyanate series produced positive reactions to toluene females attending the clinic. Forty per cent of the patients had diisocyanate and diphenyl methane diisocyanate. The patient had hand eczema for more than 2 years. Nonspecific eczema avoided further contact with isocyanates and has had no recur- was the commonest morphological pattern observed in 62 rence to date. Isocyanates are contained within polyurethane patients followed by pompholyx in 14, housewife’s eczema in resins and are used to make elastomers (e.g. coatings and 10, hyperkeratotic in nine and fingertip eczema in five. Sixty- moulds), adhesives, and rigid or flexible foams. They are five patients had one or more positive patch tests to the Indian well-known sensitizers but more commonly affect the respira- standard series with a total of 127 positive patch tests. tory system, such that strict precautionary measures are taken Potassium dichromate was the most common allergen in 25%, during contact. This has meant that contact dermatitis is rela- followed by fragrance mix in 16% and nickel sulphate and tively rare (Goossens A, Detienne T, Bruze M. Occupational para-phenylenediamine (PPD) in 14% and 13%, respectively. allergic contact dermatitis caused by isocyanates. Contact Derma- Forty-three per cent of our patients were atopics. Atopy was titis 2002; 47: 304–8). When reported in the literature, isocy- observed in up to 79% patients with pompholyx and in 51% anate allergy is most commonly seen in industrial or patients with nonspecific eczema. In India there are no studies laboratory settings, although it has previously been reported on hand eczema, its morphological patterns and the allergens in two sculptors using polyurethane resins, as in our case involved in its causation. We observed a male predominance (Militello G, Sasseville D, Ditre C, Brod BA. Allergic contact in our study and the age of involvement was similar to that dermatitis from isocyanates among sculptors. Dermatitis 2004; reported in Western studies. As in several European studies, 15: 150–3). patients were mostly engaged in occupations traditionally involving wet work and exposure, namely masonry and indus- trial work. Housewives formed a large group of our female patients presenting with hand eczema. Nonatopics were as predisposed to hand eczema as atopics and also atopic and CD-15 nonatopic patients of hand eczema had no difference in their Allergic contact dermatitis to leather phylacteries disease severity. (tefillin): a problem in the faithful A. Friedmann and P. Goldsmith Royal London Hospital, London, U.K. We report the cases of two orthodox Jewish men who devel- oped allergic contact dermatitis to the chromate in leather CD-14 straps they used during prayer. The first was a 60-year-old Acute vesicular contact dermatitis to isocyanates rabbi and the second, a 17-year-old boy. Both had atopic in an artist eczema. Each presented with worsening and uncontrollable E. McGrath and J.E. Sansom eczema chiefly on the left forearm. In addition the Bristol Dermatology Centre, Bristol, U.K. rabbi had eczema on the forehead and the back of the head. A 24-year-old self-employed artist presented to our depart- On further questioning about potential causes of their prob- ment with dramatic facial swelling and crusted erythema. She lem they revealed that they used phylacteries (or tefillin) on a had initially been seen in the accident and emergency depart- daily basis as part of their ritual prayer. The phylacteries were ment with a widespread blotchy rash, swollen face and strapped around the left arm and the head. Both patients also difficulty breathing. She had been prescribed antihistamines regularly wore large felt hats with leather linings. The and oral steroids for presumed penicillin allergy but over the 60-year-old had, in addition, coated his hat leather with a

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 88 British Contact Dermatitis Society resin containing colophony in an attempt to protect his skin. palpation she was extremely tender. The purpuric margin Patch testing to the European Standard Battery, the medica- advanced 0Æ5 cm beyond the initial skin markings in three ments and steroid series, the patients’ phylacteries and parts of hours. It was felt the appearance was in keeping with necro- the hats’ lining revealed positive reactions to chromate and tizing fasciitis and the plastic surgical team performed an the phylacteries in both patients and to colophony and his hat extensive emergency debridement. Full blood count, urea, band in the rabbi. Both patients’ contact dermatitis improved electrolytes, liver function and coagulation screen were nor- when chromate-free phylacteries were obtained, and the rabbi mal. C reactive protein was 19 mg L–1. D-dimer and fibrin- bought a new hat. Phylacteries are leather boxes containing ogen were raised. Histopathology demonstrated coagulative holy parchment that are worn every morning except on the necrosis in some areas of the dermis whilst other areas had Sabbath and holy days by observant Jewish men. They are minimal acute inflammation. There was striking thrombus for- secured to the left arm and to the head by leather straps. The mation in blood vessels. The appearances were not typical of straps are wound around the forearm and hand tightly in a necrotizing fasciitis. Gram and PAS stains were negative as was spiral fashion, or around the head in the coronal plane. We tissue culture. Subsequent investigations including protein postulate that our patients’ atopic eczema predisposed them to C and S, antithrombin III, coagulation screen, Factor V Leiden, be sensitised to the chromate in the leather straps. The add- prothrombin gene mutation, paroxysmal nocturnal haemo- itional trauma of the tight binding was probably relevant. The globinuria screen and antinuclear antibodies were negative. colophony allergy of the rabbi was clinically relevant as his Lupus anticoagulant was initially positive but later became forehead dermatitis resolved once he stopped wearing his old negative. Patch test readings at 96 h showed ++ reaction to hat with the resin coated band. It is possible that the 17-year- Xepin cream as is, ++ to doxepin hydrochloride 5% and no old boy’s hair protected him from the leather of his hat band. reactions to other constituents of the cream. Testing of nine There have been several cases of tefillin dermatitis, predomin- control subjects with doxepin hydrochloride 5% was negative. antly from Israel and the U.S.A. (Ross B, Brancaccio RR. Aller- Doxepin is a known cutaneous sensitizer. Reactions usually gic contact dermatitis to religious articles. Am J Contact Dermatitis manifest as a dermatitis but there is one reported localized 1994; 5: 160–1. Abstract; Trattner A, David M. Tefillin derma- purpuric eruption (Bonnel RA, La Grenade L, Karwoski CB, titis. J Am Acad Dermatol 2005; 52: 831-3). This low number Beitz JG. Allergic contact dermatitis from topical doxepin: may be due to the fact that the tefillin are worn for short peri- Food and Drug Administration’s postmarketing surveillance ods only – an average of an hour a day. To date this is the experience. J Am Acad Derm 2003; 48: 294–6). Purpuric contact first report of tefillin dermatitis in U.K.-based Jews. reactions have been reported with PPD, IPPD and textile dyes, although are uncommon. We believe this dramatic presenta- tion was due to an unusual contact allergy to doxepin hydro- chloride. The appearance mimicked necrotizing fasciitis, a life threatening surgical emergency. We are unaware of previous CD-16 reports of contact allergy with such a dramatic presentation. Allergic contact dermatitis to doxepin presenting acutely with haemorrhagic skin necrosis mimicking necrotizing fasciitis R.M.R. Hearn, M.S. Lewis-Jones, C.M. Green, G. Lowe, A. Munnoch,* D. Meiklejohn and S.L. Edwards CD-17 Department of Dermatology and Photobiology,*Department of Plastic Allergic contact dermatitis to English ivy Surgery, Department of Haematology and Department of Pathology, (Hedera helix) Ninewells Hospital, Dundee, Scotland, U.K. A. Hafejee, J. Williams, C. Lovell,* J.E. Sansom and A 39-year-old woman had a two year history of pruritus vul- M.H. Beck vae. Initial treatment with topical corticosteroids was followed Hope Hospital, Manchester, U.K.; *Royal United Hospital, Bath, U.K. and by Xepin (doxepin hydrochloride 5%, CHS, Norwich, U.K.) Bristol Royal Infirmary, Bristol, U.K. cream, introduced as a steroid sparing agent and anti-pruritic. English ivy (Hedera helix) is a member of the Araliaceae family. Seven months later, she presented with a 12-hour history of a It grows extensively in gardens and also covers walls and fen- rapidly progressing purpuric eruption affecting her vulva, ces. Exposure therefore occurs particularly with gardening and perineum and groin associated with severe pain and pruritus. the removal of ivy from walls. Two constituent compounds, She denied any systemic symptoms. She had applied doxepin falcarinol and didehydrofalcarinol are known irritants and cream the evening before the rash developed but denied using moderate sensitizers. We report a series of seven cases, who any other topical agents. She had a background medical his- were identified on the basis of a history of dermatitis follow- tory of atopy. Temperature was 37Æ9 C, heart rate 120 and ing exposure to English ivy and positive initial patch test reac- blood pressure 156/96. General examination was unremarka- tions to the ivy leaf. Further patch tests were performed to ivy ble. Examination of the vulva revealed oedematous and blis- extracts isolated in 1982 and 2004. These were falcarinol tered labia majora with dark purple discolouration extending 0Æ5%, didehydrofalcarinol 0Æ3% and a mixture of 75 : 25% to the lower abdominal wall, perineum and inner thighs. On falcarinol : dehydrofalcarinol 0Æ1% in petrolatum. In our series

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 British Contact Dermatitis Society 89 six of the seven patients had positive patch test results to ivy positive patch test reactions (++ or +++) to SL were 15 times leaf or extract. Four of the five patients tested to ivy leaf more common in the London cohort (0Æ9%) compared with extract were confirmed to be allergic to falcarinol from either the Singaporean cohort (0Æ06%) (P <0Æ001, v2 test). There- or both sources. Two patients had positive patch tests to the fore, our results show that the incidence and severity of SL newer didehydrofalcarinol extract provided but one of these sensitivity are significantly lower in a selected Asian popula- proved negative to patch testing with the older extract. Falcari- tion. We postulate that the consumption of chrysanthemum nol appears to be the major allergen implicated and we sug- tea since childhood in East Asia may result in induction of oral gest that screening with falcarinol should be considered in tolerance to SL, contributing to the statistically significant cases of suspected plant dermatitis. A patch test concentration lower sensitization rate in Singapore. Chrysanthemum tea is a of 0Æ03% has been recommended. The positive reactions herbal infusion popular in East Asia but is not commonly obtained with the older ivy extract demonstrate the stability of available in Europe. Allergy to Compositae is also rare in the allergen. Didehydrofalcarinol is a weaker irritant and sensi- Japan, where chrysanthemum leaves and flowers are com- tizer; it has been recommended that it could safely be used in monly eaten. Although differences in the frequency of prac- patch tests at 1% concentration. It is important to remember tices such as gardening may be relevant, we postulate that a that other sources of previous exposure to falcarinol may be higher consumption of chrysanthemum tea in the Asian popu- relevant, for example falcarinol is a constituent in some com- lation, resulting in oral tolerance, may play a role. mercial anticellulite creams, shampoos and cough expecto- rants. Concentrations in these products may produce sensitization. Several members of the Apiaceae family, notably carrots, also contain falcarinol; hence sensitized individuals should avoid contact with these, although ingestion is prob- CD-19 ably safe. Falcarinol is a bioactive polyacetylene which is cyto- Is there a need for clothing dyes in the standard toxic to cancer cells and may contribute to the health benefits series? of carrots in the diet. S. Hann, M. Hughes and N. Stone Department of Occupational Dermatology, Royal Gwent Hospital, Newport, South Wales, U.K. Clothing dye allergic contact dermatitis (ACD) classically pre- sents with a flexural pattern of eczema. The absence of clo- CD-18 thing dyes in the standard series means that patients with less Sesquiterpene lactone sensitivity variation in an typical patterns of clothing dye allergy may be underdiag- Asian vs. a European population: an effect of nosed. Clothing dyes are either azo (disperse) or anthroqui- chrysanthemum tea? none dyes. Para-phenylenediamine (PPD) is the only dye in R. Mak, I.R. White, J.M.L. White, J. McFadden and the current European standard series. It is one of the para- A. Goon* amino compounds, classically used in permanent hair dye. Department of Cutaneous Allergy, St John’s Institute of Dermatology, London, There is some cross reactivity, due to structural similarity, U.K. and *National Skin Centre, Mandalay Road, Singapore between the para-amino dyes and disperse dyes. In particular, Sesquiterpene lactones (SLs) are an important cause of allergic disperse orange 3 (DO3) is a disperse dye which degrades to plant contact dermatitis with marked geographical variation. form PPD in the skin. PPD has therefore traditionally been SLs are found in the Compositae family of plants, which is the used as a marker for both azo and para-amino dye allergy. We second largest family of flowering plants in the world. SL sen- aimed to find out whether the addition of some common dis- sitivity is far more common in America, Europe and India but perse dyes to the standard series would increase the identifica- is uncommon in the Far East. We therefore investigated its tion of patients with clothing dye ACD who would otherwise geographical difference and compared sensitization rates to SLs have been missed. Over a 2-year period we added DO3, dis- from patch tests of 6172 patients in the National Skin Centre perse blue (DB) 106 and 124, disperse yellow 3 (DY3) and in Singapore and 7557 patients in the Department of Cutane- disperse red 1 (DR1) to our standard series. One thousand ous Allergy at St John’s Institute of Dermatology in London and twenty-four patients were patch tested with the standard between January 1998 and December 2004. SL mix 0Æ1% in series and additional disperse dyes. Patients with positive reac- petrolatum (Chemotechnique, Malmo, Sweden) was used in tions to PPD and/or the additional dyes were identified. the standard series of both centres. Patch testing was per- Results were analysed for distribution of disease, series tested formed according to the ICDRG recommendations. The and the relevance of their results. Of the 1024 patients, 55 strongest readings at day 2 and 4 were recorded as +, ++ or (5Æ37%) tested positive for PPD and/or the additional disperse +++. We found that the sensitization rates to SL mix 0.1% dyes. PPD was the most common allergen, positive in 38 pet. were significantly lower in the Singaporean cohort (0Æ3%) patients (69%). In 20 patients (36%) this was associated with 2 compared with the London cohort (1Æ4%) (P <0Æ01, v test). a reaction to one or more disperse dyes (the majority to The differences were similarly seen in males (0Æ3% vs. 1Æ7%) DO3). Twenty-one (38%) patients tested positive to DO3. 2 and females (0Æ4% vs. 1Æ2%) (P <0Æ01, v test). Strong Only two of these patients reacted to DO3 in the absence of a

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 90 British Contact Dermatitis Society reaction to PPD. The next most common positive reactions DMAPA in eight patients. In contrast with a previous study of were to DB124 and DB106 (26% and 27% respectively). Only similar size (Angelini G, Foti C, Rigano L et al. 3-dimethylami- two of these patients were concomitantly allergic to PPD. DY3 nopropylamine: key substance in contact allergy to cocamido- was the next most common positive (14%) and DR1 the least propyl betaine? Contact Dermatitis 1995; 32: 96–9), only a common (13%). Our results were consistent with other minority of our patients (9) reacted to both DMAPA and reports of the most prevalent dye reactions and cross reactivity cocamidopropyl betaine. Of the 47 patients who reacted to (Koopmans AK, Bruynzeel DP. Is PPD a useful screening DMAPA, the face was affected in 38. Our results show that agent? Contact Dermatitis 2003; 48: 89–92). Almost half our DMAPA is a common cause of positive patch test reactions cases of positive reactions to clothing dyes would have been and indicate that it should be included when patch testing missed if PPD alone had been used as a marker of clothing patients suffering from facial eczema. dye ACD. DO3 was very strongly linked to PPD, rarely react- ing alone. There would be little benefit from including it on the standard series. DB124 and DB106 are the next most com- mon reactors in our series. They reacted independently of PPD in the majority of cases. We would therefore support the CD-21 inclusion of DB124 and DB106 in the standard series. Allergic contact dermatitis to benzophenone-4 in a printer T. McPherson and S. Cooper Department of Dermatology, Churchill Hospital, Oxford, U.K. A 19-year-old printer presented with a history of a facial ery- CD-20 thema at the end of a working day in January. That day she Dimethylaminopropylamine: an important facial had been using inks that she did not normally use for a one- allergen? off print. The facial erythema evolved over the subsequent S. Abdul Ghaffar, H. Horn and R.D. Aldridge days, with scale and severe pruritus. There was no evidence of The Department of Dermatology, The Royal Infirmary of Edinburgh, angio-oedema or respiratory compromise. She had a previous Edinburgh, U.K. history of mild atopic eczema but no history of sunscreen Dimethylaminopropylamine (DMAPA) is a known impurity of problems. Patch testing was performed to BCDS standard ser- cocamidopropyl betaine, an amphoteric surfactant present in ies, cosmetics, photo-patch series and (meth)acrylates. Benzo- many shampoos, hair dyes, toothpastes, soaps and other toilet- phenone-4 (2-hydroxy-4-methoxy benzophenone-5-sulfonic ries. DMAPA has been considered an unimportant contact acid) was positive (++) on both covered and irradiated allergen (McFadden JP, Ross JS, White IR et al. Clinical allergy (5 J cm–2) sites. Subsequent investigation revealed the pres- to cocamidopropyl betaine: reactivity to cocamidopropylamine ence of the sunscreen benzophenone in the phototext lacquer and lack of reactivity to 3-dimethylaminopropylamine. Contact (UV matt) that she had used the day she developed the rash. Dermatitis 2001; 45: 72). Unlike cocamidopropyl betaine, it is Her normal printing lacquers did not contain this sunscreen. It not included in the facial series currently recommended by was not clear whether she was sensitised from previous use of the British Contact Dermatitis Society. As it was our impres- sunscreen or previous exposure to this lacquer. Benzophenones sion that DMAPA is a common cause of positive patch test are UV absorbers. They may cause photo-allergic or allergic reactions, we audited our patch test results to determine the contact dermatitis from their presence in sunscreens. However numbers of patients reacting to DMAPA and cocamidopropyl this case highlights the fact that they are increasingly being betaine. Between 1 May 2000 and 31 December 2006, 1919 used in products other than traditional sunscreens. There are patients were patch tested to an extended Chemotechnique previous reports of facial erythema as a result of exposure to (Malmo¨, Sweden) cosmetic series, which included DMAPA benzophenones in cosmetics and shampoos and some indus- (1% aqueous), and cocamidopropyl betaine (1% aqueous). trial products. Benzophenones are included in paints and There were 598 clinically relevant positive reactions at 96 fluorescent lacquers to inhibit degradation of colours. Derma- hours. The five most common allergens were Me-isothiazoli- titis is frequently reported in printers although most com- none (61 patients), cocamidopropyl betaine (55 patients), Eu- monly the reaction is irritant. This is an unusual case of xyl K 400 (48 patients), Quaternium 15 (42 patients) and allergic contact dermatitis to benzophenone-4 as a component DMAPA (39 patients). Nonrelevant reactions to cocamidopro- of a printing dye. The importance of this as an occupational pyl betaine were seen in an additional 14 patients and to risk should be considered.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 British Contact Dermatitis Society 91

Posters

CD-22 CD-23 The investigation of allergic skin disease: both patch Audit of patch testing in Merseyside and North and prick testing are essential Wales N. Usmani and M. Wilkinson N. Hashim, G. Hill* and C. King Leeds General Infirmary, Leeds, U.K. Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, In our clinic we routinely patch test patients referred from the Merseyside, U.K. and *North East Wales NHS Trust, Wrexham, Wales, occupational health service for the investigation of contact U.K. urticaria from latex. We also undertake both patch and prick An audit was conducted in 2004 to determine the variability tests (where indicated) in patients referred with persistent in practice and organization of patch test (PT) clinics within dermatitis or eczema. Prick testing is currently undertaken by the Merseyside and North Wales region. Results were com- dermatologists, allergists and immunologists. If investigation pared with the minimum standards set by the British Associ- of allergic skin disease is carried out by a nondermatologist it ation of Dermatologists (BAD) guidelines (Bourke J, Coulson is highly unlikely that patch testing will be performed. We I, English J. Br J Dermatol 2001; 145: 877–85) and with an undertook a retrospective analysis of patients who had been audit conducted in 2001, based on the then draft BAD guide- prick tested to establish if an incomplete diagnosis would have lines. Questionnaires were sent to eight centres and six ques- been reached if patch testing had been omitted. Details of tionnaires were returned (75% response rate). The patients who had attended for patch testing between July respondents were consultants (C) (50%), staff grade doctors 2004 and December 2005 were analysed. Patients who had (SG) (33%) and associate specialists (AS) (17%). Five centres had prick tests and patch tests were identified. The outcomes (83%) have a dedicated PT clinic. The average waiting time of prick tests and patch tests were documented together with for a PT appointment was 4Æ4 months, with an average of the clinical relevance. Results showed that 330 of 1060 5.25 patients investigated per week. The average PT rate was patients referred to the clinic were prick tested. Of these 1 : 2079 of the population served. All centres provided infor- patients, 54% were referred from dermatologists and 27% mation leaflets before testing. C, SG and AS doctors with a from the occupational health service. Positive patch test results major interest in PT saw patients for the initial consultation were found in 45% of these patients, which were of relevance and prescribed the appropriate patch tests. They also per- in 35%. Positive reactions on prick testing were seen in 68 formed readings; however, nurses performed the first reading patients of whom 36 had positive patch tests (53%), relevant at three centres. Most (83%) centres took readings on days 2 in 32 patients (47%). Nine of 106 health workers referred to and 4, and have the facility to take additional readings at a exclude latex contact urticaria had positive prick tests to latex. longer interval. C, SG and AS doctors determined the relevance Of these patients, 50 demonstrated delayed type hypersensitiv- of the final PT results and discussed the conclusions with the ity. Nickel, cobalt, rubber and its additives were the most patients. All centres used the BCDS standard battery routinely. common allergens in this patient group. Of the 262 patients The number of extended batteries varied (range 14–22); four who had negative prick tests 121 had positive patch tests centres (67%) frequently test patients’ own products. All cen- (46%), relevant to the history in 108 patients (41%). Whilst tres have reference books and journals on occupational and none of the six patients referred for investigation of reaction contact dermatitis available in the department. All centres also to local anaesthetics had a positive prick test, one was allergic have a dedicated area for the preparation and storage of aller- to local anaesthetic on patch testing. In conclusion we have gens, which are refrigerated. Three centres performed photo- shown that omission of patch testing from the investigation of patch testing and three centres performed prick testing rarely. allergic skin disease, even when contact urticaria may be the Four centres (67%) have access to an immunologist; however, sole suspected diagnosis, would result in the frequent missed in only one centre is the service available in the same Trust. diagnosis of contact allergy. We therefore recommend that All centres provided information leaflets produced either by patients with suspected allergic skin disease are investigated in the BCDS or the respective departments regarding confirmed an environment where investigation of both immediate and allergens. The average counselling time on conclusion of PT delayed type hypersensitivity can be undertaken. was 18 minutes. Only one centre recorded results on a data- base and none of the centres regularly review or audit their results. To our knowledge this is the first reported regional re-audit assessing compliance with the BAD guidelines. In conclusion, there has been a greater uniformity in practice across the region since the previous audit and the group were compliant with the minimum standards set down by the BAD,

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 92 British Contact Dermatitis Society apart from the suggested rate of patch testing. Areas identified CD-25 as requiring further improvement included waiting times and Diisopropranolamine (DIPA) induced contact data collection, preferably using the BCDS database. dermatitis in a football referee A.S. Belgi and M.M.U. Chowdhury Welsh Institute of Dermatology, University Hospital of Wales, Cardiff, U.K. A 45 year-old part time football referee developed an erythe- matous itchy scaly eruption on his right knee within minutes CD-24 of using topical ibuprofen gel underneath a neoprene rubber It’s not only latex. Nitrile gloves can produce aller- support. His skin eruption became generalized involving his gies too other leg and trunk within a few days of the initial presenta- T. McPherson and S. Cooper tion. He had a history of deodorant allergy. Patch tests to the Department of Dermatology, Churchill Hospital, Oxford, U.K. standard battery, neoprene support and to ibuprofen gel Awareness of latex allergy has prompted widespread use of a revealed 1+ reaction to Fragrance mix II and 2+ reaction to variety of alternative glove materials. There are now increasing ibuprofen gel. Fragrance mix allergy was in keeping with his numbers of cases of allergies developing to the ‘hypoallergen- history of deodorant intolerance. He had further patch testing ic’ alternatives. We present two cases of allergic reactions to with the ingredients of ibuprofen gel including ibuprofen and nitrile gloves. Patient 1: A kitchen porter presented with a DIPA. Patch test to ibuprofen 1% and 5% in petrolatum was very severe hand dermatitis following use of latex gloves. He negative but patch test to both 1% and 5% DIPA in petrolatum changed to nitrile gloves without any improvement in his was 2+ positive. All eight control subjects subsequently tested eczema. Patch testing to the BCDS standard and medicament with 1% DIPA showed negative patch tests. DIPA is an organic series plus an extended rubber series confirmed positive reac- base used as a neutralising agent in cosmetics and toiletries. tions to rubber chemicals; zinc-diethyldithiocarbamate, zinc- There are about eight case reports in the literature of DIPA dimethyldithiocarbamate and thiuram mix. He also had a 2+ allergy and most of them developed contact allergy after using reaction to a piece of his own nitrile glove. Prick tests to latex topical NSAID gel. In 1973 Cronin reported a young female and nitrile glove were negative. Ingredient listings for the with contact dermatitis secondary to DIPA in her eye gloss nitrile glove were given by the manufacturer and confirmed (Cronin E. Contact Dermatitis. Edinburgh: Churchill Livingstone, the presence of zinc-diethyldithiocarbamate, a rubber acceler- 1980; 114). Although ibuprofen itself can cause contact ator. This explained his allergic contact dermatitis to nitrile allergy, a positive patch test to DIPA in our patient as well as gloves. Patient 2: A 24-year-old F2 doctor working in anaes- the negative patch test results in controls confirmed DIPA thetics gave a history of tingling, erythematous weals on the allergy in our patient. As DIPA is an ingredient of topical NSAID back of the hands extending to the forearms within minutes preparations, DIPA allergy should be suspected in patients with of wearing latex gloves. After switching to nitrile gloves history of contact allergy to topical NSAID preparations. exactly the same symptoms occurred. Patch testing to the standard, medicament and extended rubber series were nega- tive. Prick testing to latex (Soluprick 10 Hep, ALK-Abello´ A/S, Hersholm, Denmark) was positive and prick testing was also positive through a nitrile glove, with a weal larger than the CD-26 positive control. Further investigation of the cause of the nitri- Allergic contact dermatitis to topical mepyramine le type 1 reaction was hindered by the manufacturer’s reluc- maleate tance to supply ingredients of the nitrile glove for testing. G. Kaushal and D. Orton Allergic contact dermatitis and type 1 hypersensitivity to nitri- Amersham Hospital, Amersham, U.K. le gloves have only rarely been reported to date. However, we Topical antihistamines are commonly sold as over-the-counter believe that problems with nitrile gloves may be more fre- products for the treatment of insect bite reactions. Anthisan quent than previously appreciated. It is important to remem- cream has been available on the U.K. market for approxi- ber that rubber chemicals (in particular accelerators such as mately 40 years. A 36-year-old woman presented with a his- thiurams, benzothiazoles and carbamates) may also be present tory of multiple insect bites during the summer. After treating in synthetic rubber gloves. As ‘hypo-allergenic’ gloves gain them with Anthisan cream she developed an eczematous rash increasing popularity over latex gloves more frequent reports within 48 hours at the sites of product application. There was are to be expected of both allergic contact dermatitis and no previous history of eczema or atopy. The eczema resolved immediate hypersensitivity to rubber additives. with oral and topical steroids. Subsequent patch tests revealed allergic contact dermatitis to nickel, and Anthisan cream applied ‘as is’. The active ingredient antihistamine was sourced and further patch tests performed. She was found to be aller- gic to mepyramine maleate (MM) (2% pet). Patch tests to MM were negative in control subjects. MM is an ethylenediamine derivative, and defined as a first generation

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 British Contact Dermatitis Society 93 antihistamine known for its antimuscarinic properties. Morgan ropine and streptomycin. On awakening the next morning he reviewed 30 000 cases of whom only 21 had developed sensi- had a generalized erythematous pruritic rash. He returned to tivity to topical antihistamines (Morgan JK. Iatrogenic epider- the U.K that day and went to the hospital from the airport. mal sensitivity. Br J Clin Prac 1968; 22: 261–4); this is less On admission, he was febrile and had a florid erythroderma than one-thousandth of a percentage. Baker stated that the risk affecting every part of his body, except the area previously of sensitization to topical antihistamines is small especially in covered by his bathing trunks. This area was completely normal as opposed to eczematous skin (Baker H. Itch (Letter). spared with a striking demarcation line at the transition. He Lancet 1980; 2: 863). In this patient, there was no pre-existing also had generalized oedema and extensive bullae on his trunk skin condition. MM has a similar chemical structure to clemas- and the inner aspects of his arms and legs. A skin biopsy tine, and the same salt compound as chlorphenamine. Cross- showed marked papillary oedema and a perivascular chronic reactivity is a possibility, but a Medline search did not reveal inflammatory infiltrate with scanty eosinophils extending to any data to support this, nor does the manufacturing company the mid-dermis. Review of his notes revealed that he had a know of any such cases. This case highlights that even rela- history of chronic otitis externa. Patch testing in 2000 had tively inert compounds used in the treatment of ‘allergic’ reac- revealed a +++ blistered positive reaction to clioquinol, tions can still rarely cause sensitization in patients. attributed to his long-term use of Locorten-Vioform ear drops (Amdipharma, Basildon, U.K.) and Betnovate C (GSK, Uxbridge, U.K.) ointment. He had been advised to avoid cli- oquinol and its related compounds in both topical and system- ic forms. This patient was known to be strongly allergic on CD-27 patch testing to clioquinol. He was subsequently exposed sys- Systemic allergic contact dermatitis from a temically to a cross-reacting drug, namely diiodoquinol, and quinolone suffered a severe erythrodermic reaction with bullae. We pos- R. Ramesh, F. Tatnall and M. Murdoch tulate that there was Koebnerization into the sunburnt skin to Watford General Hospital, Watford, U.K. explain the sparing of the area covered by his bathing trunks. A 58-year-old man presented with erythroderma to the Acci- Diiodoquinol is a compound closely related to clioquinol, and dent and Emergency department, immediately after returning cross reactions amongst the quinolones are well recognized. from a holiday in Egypt. Whilst in Egypt he had become very Systemic allergic contact dermatitis with the quinolones has sunburnt, with only the area covered by his bathing trunks been described (Cronin E. Medicaments. In: Contact Dermatitis. spared. Following the sun exposure he had become unwell Edinburgh: Churchill Livingstone, 1980; 220). There are with vomiting. A local doctor attended him and prescribed major concerns about the safety of halogenated hydroxyquino- three oral doses of Streptoquin. Streptoquin is a combination lines due to their neurotoxic effects and indeed their use has drug comprising diiodoquinol, phthalylsulphathiazole, homat- been banned in some countries.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 81–93 British Society for Dermatopathology: Summary of Papers

DP-1 deposition in nephrogenic fibrosing dermopathy. J Am Acad Nephrogenic fibrosing dermopathy following Dermatol 2007; 56: 27–30; High WA, Ayers RA, Chandler repeated MRI using gadolinium contrast media J et al. Gadolinium is detectable within the tissue of patients A. Gulati, C. Harwood, N. Ashman, R. Cerio and with nephrogenic systemic fibrosis. J Am Acad Dermatol 2007; C. Proby 56: 21–6) and on December 21st 2006 the FDA advised health Barts and the London NHS Trust, London, U.K. care professionals not to use gadolinium-based contrast agents Nephrogenic fibrosing dermopathy (NFD), first described in in patients with moderate to end-stage kidney disease. It is 2000 (Cowper SE, Robin HS, Steinberg SM et al. Scleromyxo- imperative to recognise NFD and commence treatment early as edema-like cutaneous diseases in renal-dialysis patients [Letter]. current therapeutic strategies may halt progression of disease, Lancet 2000; 356: 1000-1), is a new disease afflicting individu- but are not able to reverse the disease process significantly. als with impaired renal function. Over 200 cases have been reported, although it is likely that more cases may be unrec- ognized. NFD is a fibrosing disorder characterized by dermal thickening and oedema. It resembles scleroderma clinically and scleromyxoedema histopathologically. It produces harden- ing of skin, initially of the extremities spreading proximally DP-2 and often resulting in contractures with significant impairment Animal type melanoma: malignant or benign? of mobility and quality of life. In 5% of cases, a fulminant A. Alam, G. Osborne and J. Leonard form progresses rapidly to death. Recent evidence with St Mary’s Hospital, Paddington, London, U.K. important clinical implications suggests gadolinium may be Animal type melanoma is a rare histological subtype of mela- the aetiological agent. We present a 36-year-old man with noma similar to a variant originally described in horses. The Von Hippel Lindau syndrome, who had bilateral nephrectom- exact nature and malignant potential of these tumours remains ies for renal squamous cell carcinoma in 2002 and renal trans- unclear and hence controversy exists in the diagnostic criteria plantation in 2005. He had cerebellar haemangiomas resected and terminology. Characteristically there is usually a dermal in 1991, 2001 and 2003. As a consequence, he has had mul- proliferation of deeply pigmented elongated or rounded cells, tiple magnetic resonance imaging scans (MRI) using gadolin- hence the alternative name of pigment synthesizing mela- ium as contrast agent during a period when he was receiving noma. Histological diagnosis presents challenges, with differ- haemodialysis. He has experienced progressive difficulty using ential diagnoses including cellular blue naevus, malignant blue his limbs since 2004 and his symptoms noticeably worsened naevus, deep penetrating naevus, plexiform spindle naevus, immediately after he underwent two MRI in one day. He now pigmented spindle and epithelioid naevus and regressed mela- has indurated skin of his limbs, contractures of his fingers, noma with prominent melanophages. Tumours generally show fixed flexion deformities of his knees and marked proximal mild to moderate atypia and a low mitotic rate. Interestingly, muscle wasting in both legs. NFD is a diagnosis that relies prognosis appears to be better than in superficial spreading heavily on clinicopathological correlation. Histopathologically, and nodular malignant melanomas. Even in the presence of NFD manifests with a proliferation of dermal fibroblasts and nodal metastases, reports of mortality are few, hence the sug- dendritic cells, thickened collagen bundles, increased elastic gestion of the alternative term, pigmented epithelioid melano- fibres and mucin deposition, although early changes may be cytoma. In recent studies, some authors have suggested that very subtle. Biopsy, in this case, was consistent with NFD. animal type melanomas are identical to epithelioid blue naevi Extensive investigation has not identified any other cause for found in patients with Carney complex, a rare genodermatosis his symptoms. He has not responded to thalidomide or renal associated with PRKAR1A gene mutations and most commonly transplantation, which have been reported to be beneficial in featuring cardiac myxomas, cutaneous myxomas, spotty some cases. He is currently referred for extracorporeal photo- pigmentation of the skin, endocrinopathy, and both endocrine pheresis with ongoing physiotherapy. The recent emergence and nonendocrine tumours. We present a review of the cur- of NFD suggests a novel agent or intervention is involved in rent literature and a case of a 30-year-old City financier with its pathogenesis and a plethora of recent reports now implicate an animal type melanoma of the back with a Breslow thick- gadolinium, particularly if used in the context of metabolic ness of 3Æ6 mm and with bilateral positive axillary sentinel acidosis. Two series have identified gadolinium within lymph nodes, illustrating the difficulties in diagnosis, manage- involved tissues (Boyd AS, Zic JA, Abraham JL. Gadolinium ment and prognostication in these tumours.

2007 The Authors 94 Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 British Society for Dermatopathology 95

DP-3 DP-4 A case series of cutaneous carcinosarcoma Naevus cell aggregates in sentinel lymph node R. Rose, W. Merchant,* G. Stables, C. Lyon and parenchyma: a potential diagnostic pitfall with A. Platt malignant melanoma: the Royal Free Hospital Department of Dermatology, The General Infirmary at Leeds, U.K.; experience *Department of Pathology, The General Infirmary at Leeds, U.K.; M. Singh, A. Robson,* E. Calonje and F. Deroide Department of Dermatology, York District Hospital, U.K. and Royal Free Hospital, London, U.K. and *St John’s Institute of Dermatology, Department of Plastic Surgery, Castle Hill Hospital Hull, U.K. St Thomas’ Hospital, London, U.K. Primary cutaneous carcinosarcoma is exceedingly rare with The prognostic value of detecting micrometastases within senti- just over 50 reports in the literature; a further five cases are nel lymph nodes (SLNs) of patients with malignant melanoma presented here. The following patients were all male with an is well established. A recent study (Morton DL, Thompson JF, age range of 58–84 years (Table 1). All lesions were thought Cochran AJ et al. Sentinel-node biopsy or nodal observation in to represent basal cell carcinomas at initial presentation. None melanoma. N Engl J Med 2006; 355: 1307–17) suggested that of the cases are known to have metastasized. Carcinosarco- patients with intermediate thickness melanomas (1Æ2–3Æ5 mm) ma is a rare tumour comprising an epithelial and a mesen- and positive SLNs derive a survival advantage from immediate chymal component, both of which are malignant. completion lymphadenectomy. It is well recognized that naevus Carcinosarcomas of visceral tissue are more common and may cells (usually HMB-45 negative) can be found within the cap- affect the adrenals, colon, endometrium, lungs and prostate sule and trabeculae of lymph nodes. However it is less well gland. Tumours from these sites often develop metastases and known that naevus cells can also be located within the lymph prognosis is poor. Primary cutaneous carcinosarcomas usually node parenchyma. This situation poses a diagnostic challenge as behave less aggressively than their visceral counterparts, tend- the location of the cells within the parenchyma may wrongly ing to present on the sun-exposed skin of older patients. The suggest a diagnosis of metastatic melanoma, with patients risk of metastasis is higher if the epithelial component is squa- undergoing unnecessary completion lymphadenectomy with its mous cell carcinoma rather than basal cell carcinoma and in attendant morbidity. We performed a retrospective study to lesions more than 2 cm in diameter which are longstanding identify the frequency of naevus cell aggregates within SLNs or have undergone recent growth. The most commonly repor- from 2003 to 2006 at the Royal Free Hospital. We identified 46 ted epithelial component is squamous cell carcinoma and then SLNs in 39 patients which showed intranodal cells with positive basal cell carcinoma and for the mesenchymal component it is staining for Melan-A and or HMB-45. Of the 46 nodes, 28 were atypical fibroxanthoma, osteosarcoma and then chondrosarcoma. classified as malignant lymph node deposits; nine cases as For visceral tumours this has been shown to be a metaplastic benign naevus cell deposits, characteristically located within the process from the epithelial component and hence this is the capsule or trabeculae of the lymph node; and nine problematic most likely pathogenic mechanism for cutaneous lesions (Tran cases where the immediate distinction between benign and ma- TA, Muller S, Chaudahri PJ, Carlson JA. Cutaneous carcinosar- lignant deposits was not clear. These cases have all arisen since coma: adnexal vs. epidermal types define high- and low-risk the introduction of Melan-A into our SLN protocol. Melan-A is a tumors. Results of a meta-analysis. J Cutan Pathol 2005; 32: sensitive and specific marker for both benign and malignant 2–11). melanocytes, with a higher sensitivity than HMB-45. The last three authors reviewed the nine problematic cases independ- ently. There was general concordance with four cases consid- Epithelial Mesenchymal Site Duration Signs Diameter component component Treatment ered to be melanoma, three benign, one suspicious for Temple 3 yrs Exophytic, 20 mm BCC Spindle cells Surgery melanoma and one likely to be benign but both inconclusive. overgranulating and tumour The two inconclusive results occurred with scattered cells in the giant cells Chin 4 yrs Ulcerated 8 mm BCC Pleomorphic Surgery parenchyma which were unidentifiable on the haematoxylin spindle cells and eosin stains. Two cases with intraparenchymal deposits Shoulder Noticed Nodule 6 mm BCC Osteoid Surgery incidentally metaplasia which were originally reported as melanoma were reclassified Cheek Unknown Unknown Unknown BCC Osteoid Surgery as benign naevus cells by all three pathologists on review. In metaplasia conclusion, the aim of this study is to raise awareness to the rare Inner Unknown Ulcerated Unknown BCC Spindle cells Surgery canthus and tumour existence of intraparenchymal naevus cells, and to help patholo- giant cells gists identify them. Cells located within the capsule are almost always naevus cells compared to aggregates in the peripheral sinus which are usually melanoma. When deposits occur in the parenchyma we recommend close analysis of morphological features stained with haematoxylin and eosin in correlation with HMB-45 status and the primary melanoma where possible.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 96 British Society for Dermatopathology

DP-5 DP-6 Two unusual cutaneous fungal infections in Absence of widespread nuclear betacatenin supports immunosuppressed patients a diagnosis of basal cell carcinoma with matrical S. Rajpara, P. Woo, D. Thompson* and R. Laing differentiation Dermatology Department, Aberdeen Royal Infirmary, NHS Grampian, R. Carr, S. Taibjee, A. Bedlow and A. Lazar* Aberdeen, U.K.; *Pathology Department, Aberdeen Royal Infirmary, Aberdeen, Departments of Histopathology and Dermatology, Warwick Hospital, Warwick, U.K. and Infection Unit, Aberdeen Royal Infirmary, Aberdeen, U.K. Warwickshire, U.K. and *Departments of Dermatology and Pathology, Case 1: A 60-year-old lady with a 5-year history of chronic University of Texas MD Anderson Cancer Center, Houston, Texas, U.S.A. demyelinating polyneuropathy has been on long term pred- Basal cell carcinoma (BCC) with matrical differentiation is a nisolone. She has also had azathioprine for two years followed rare tumour which must be differentiated from pilomatrical by ciclosporin for 6 months and cyclophosphamide for one carcinoma. The former appears to behave similarly to conven- year. In addition she was on intravenous immunoglobulins for tional BCC but the latter may be an aggressive tumour with the last three years. Whilst on cyclophosphamide she was significant morbidity and mortality. Betacatenin is a 92 kDa referred to the Department of Dermatology with a 4-month protein involved in the Wnt signalling pathway which has history of an enlarging, occasionally itchy lesion over her left been shown to play a critical role in the hair cycle and the forearm. Examination revealed a 2 · 1Æ5 cm nodular solitary development of both pilomatricoma and pilomatrical lesion covered with a brownish black crust. Histology showed carcinoma (Hassanein AM, Glanz SM. Beta-catenin expression granulomatous inflammation and mycological cultures grew in benign and malignant pilomatrix neoplasms. Br J Dermatol Phialophora verrucosa. Initial treatment with itraconazole 100 mg 2004; 150: 511–16). We report a case where immunohisto- twice a day for 4 months was unsuccessful, but there has been chemistry, including betacatenin, helped to support the good improvement with oral terbinafine 250 mg daily for the diagnosis of BCC with matrical differentiation. A 67-year-old last 14 months. She has always lived in the U.K. and did not woman presented with an 18 · 15 mm nodular lesion on the recall any trauma to the forearm. She was in South Africa for scalp clinically resembling a BCC. A punch biopsy showed part 2 weeks 14 years ago. Chromoblastomycosis is most fre- of a basaloid tumour with a high mitotic rate, consisting of quently observed in tropical countries and in rural workers in lobules with striking central keratinisation, ghosted nuclei and subtropical areas and occurs more frequently over the legs and trichohyaline granules typical of matrical differentiation. feet through traumatic inoculation (Esterre P, Queiroz-Telles Histological features in favour of a diagnosis of BCC included F. Management of chromoblastomycosis: novel perspectives. focal peripheral palisading, fibrosis surrounding tumour Curr Opin Infect Dis 2006; 19: 148–52). We could not find any lobules, and retraction artefact between the basaloid case of cutaneous P. verrucosa infection in the literature reported epithelium and stroma. Immunohistochemistry demonstrated from the U.K. Case 2: A 25-year-old Thai lady with HIV dis- that the basaloid epithelium was diffusely strongly positive for ease was referred to the Department of Dermatology with BerEP4 and negative for cytokeratin-7, CD34 and epithelial 2-month history of itchy, unsightly lesions over the face and membrane antigen. The nuclear proliferation marker Ki67 was neck. Examination revealed dusky erythematous plaques and strongly positive in 90% of tumour nuclei, p53 staining was nodules with excoriated lesions over the face and neck. She moderate in 50% of tumour nuclei. Betacatenin, however, was had some superficial nodular lesions on the hard palate. The focally positive in only approximately 5% of tumour nuclei. patient was poorly compliant with her antiviral treatment and Based on the histological architectural features, and supported had a CD4 cell count of 65 cells mm–3 and an HIV viral load by the largely negative betacatenin nuclear staining, we of 4Æ1 · 105 copies mL–1. The skin biopsy was consistent with favoured a diagnosis of BCC with matrical differentiation and cutaneous . The skin eruption improved with advised complete excision as for BCC. The excision specimen oral itraconazole 100 mg twice daily, but her skin flared up subsequently confirmed features of a typical nodular BCC later because of her noncompliance with treatment. Histoplas- blending with tumour lobules showing striking central matrical mosis is a rarely observed mycotic infection in Europe and is differentiation. Previous studies have shown that pilomatri- usually seen in immigrants from the other endemic countries. coma and pilomatrical carcinoma display diffuse nuclear beta- Disseminated cutaneous histoplasmosis most commonly affects catenin immunostaining, due to activating mutations in the the arms, face and trunk. It can present as macules, papules, CTNNB1 gene. This contrasts with BCC, which may show focal patches, plaques, pustules, abscesses, erysipelas-like eruptions, nuclear accumulation of betacatenin only (El-Bahrawy ulcerations, purpura or erythroderma. We could only find one M, El-Masry N, Alison M et al. Expression of beta-catenin in case in the literature of cutaneous histoplasmosis reported basal cell carcinoma. Br J Dermatol 2003; 148: 964-70). In this from the U.K. in a patient from the U.S.A. who also had sys- case, immunohistochemistry on the biopsy lent support to our temic lupus erythematosus (Price DA, Ong ELC. Cutaneous favoured diagnosis of BCC with matrical differentiation, subse- histoplasmosis in a patient with systemic lupus erythematosus. quently confirmed from the complete excision specimen. BCCs Lancet Infect Dis 2003; 3: 296). with matrical differentiation are extremely rare, and more studies are required to confirm the suggestion of relatively non aggressive biological behaviour in comparison to true pilomatrical carcinoma, its histological mimic.

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DP-7 DP-8 Primary cutaneous nerve hyperplasia: a novel Axillary granular parakeratosis clinico-pathological entity N. Attard, E. Calonje* and B. De Silva J. Lai-Cheong, P. Farrant,* E. Derrick,* P. Banerjee, Luton and Dunstable NHS Trust, Luton, U.K. and *St John’s Institute of H. du P Menage´ and E. Calonje Dermatology, St Thomas’ Hospital, London, U.K. St John’s Institute of Dermatology, London, U.K.; *Department of Dermatol- Axillary granular parakeratosis (AGP) was described in 1991 ogy, Brighton General Hospital, Brighton, U.K. and Department of as benign erythematous, hyperkeratotic pigmented lesions in Dermatology, University Hospital Lewisham, London, U.K. the axillae (Northcutt AD, Nelson DM, Tschen JA. Axillary We present two cases of a possible novel clinico-pathological granular parakeratosis. J Am Acad Dermatol 1991; 24: 541–4) entity, primary cutaneous nerve hyperplasia, which is charac- but subsequently this definition was extended to disease af- terized by nerve hyperplasia in the reticular dermis without fecting any intertriginous area. It is a rarely reported condition any evidence of known precipitating factors. Case 1 was a 65- of unknown aetiology which shows no racial predilection but year-old white Caucasian female who presented with a 2-year is commoner in women. Characteristic histopathological find- history of a painful area on her upper back. She was otherwise ings include; acanthosis, hyperkeratosis and retention of kera- well with no significant past medical history. Clinical examin- tohyalin granules in the stratum corneum. A previously well ation revealed a palpable area of linear erythema measuring 6 18-year-old female was admitted with an erythematous, con- cm in length near her neck. A lesional incisional skin biopsy fluent tender rash with no oral involvement. In the weeks showed normal epidermis with prominent nerve hyperplasia before, she had been treated with topical steroids, flucloxacil- within the reticular dermis. There was no inflammation or lin and oral terbinafine following which the initially intertrigi- granulomata. Direct immunofluorescence and special stains for nous rash became extensive with cutaneous burning, pain and amyloid including thioflavin T and Congo red were negative. erosions. The differential diagnosis included eczema, a drug Topical therapies including steroids, capsaicin and doxepin eruption and pemphigus foliaceous. Skin biopsy with haema- were ineffective. However, a small evening dose of oral ami- toxylin and eosin stain showed hyperkeratosis and retention of triptyline has been beneficial in reducing the burning sensa- keratohyalin granules in the stratum corneum. In the underly- tion present in the affected area. Case 2 was a 44-year old ing superficial dermis there was a patchy mononuclear cell Chinese female who presented with a four year history of lin- infiltrate. These findings were consistent with AGP. There was ear ‘‘lumps in the skin’’, which predominantly affected her no spongiosis or acantholysis. Fungal and bacterial cultures forearms and thighs. These lesions were largely asymptomatic. were negative. Direct immunofluorescence (IMF) showed faint Examination revealed palpable linear flesh-coloured dermal epidermal intercellular IgG. Indirect IMF showed intercellular nodules on her forearms and thighs. Lesional skin biopsy IgG deposition (titre 1 : 100) on monkey oesophagus suggest- showed intact epidermis with marked hyperplasia of the ive of pemphigus. The eruption cleared completely with oral nerves in the superficial and mid-dermis. These nerves fol- corticosteroids and topical preparations. However, some weeks lowed the path of neurovascular bundles and exhibited S100 later she developed a similar limited erythematous rash in the immunostaining. Despite the different physical appearances of inframammary area, groin, buttocks and posterior thighs. Sys- skin lesions present in the above two cases, the histological temically she was well and had received no new medication. features were similar and consisted of reticular dermal nerve A repeat biopsy was again consistent with AGP. IMF, both hyperplasia without any apparent cause. An increase in the direct and indirect, was negative. The rash settled with potent numbers of both myelinated and nonmyelinated dermal nerves topical corticosteroids and emollient treatment. The aetiology has previously been described in clinically normal skin of of AGP is not known. Possible contributory factors include patients with multiple endocrine neoplasia type 2b. Neuro- excessive use of personal hygiene products, sweating and hyperplasia is also a common feature of nodular prurigo, occlusion. At the molecular level it is postulated that a defect probably arising as a result of increased p75 low-affinity nerve in profilaggrin to filaggrin processing results in the retention growth factor receptor (p75 NGFr) expression in Schwann of keratohyalin granules in the stratum corneum (Metze D, cells and perineurium cells in cutaneous nerve components. Rutten A. Granular parakeratosis – a unique acquired disorder Another study demonstrated neural hyperplasia in several of keratinisation. J Cutan Pathol 1999; 26: 339–52). The flare in patients with chondrodermatitis nodularis helicis, which is our patients’ rash associated with transiently positive immuno- characterized by the presence of painful tender nodules on the fluorescence studies cannot be definitively explained. The helix of the ear. In this report we describe two cases in which absence of acantholysis on skin biopsy is against a diagnosis dermal nerve hyperplasia was a prominent histological feature of (drug induced) pemphigus. This case highlights AGP, a with no evidence of any causative factors. Primary cutaneous poorly recognized entity which should be included in the dif- nerve hyperplasia is thus a possible novel clinicopathological ferential diagnosis of intertriginous rashes. entity and should only be considered after known causes of nerve hyperplasia are formally excluded on clinical or investi- gative grounds.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 98 British Society for Dermatopathology

DP-9 DP-10 Intravascular histiocytosis of the right eyelid Reticular erythematous mucinosis following N. Webber, R. Russell-Jones, O. Espinosa* and mastectomy A. Robson B. Wynne and F. Powell St John’s Institute of Dermatology, Guy’s and St Thomas’ Hospitals, London, Mater Misericordiae Hospital, Dublin, Ireland U.K. and *Pathology Department, John Radcliffe Hospital, Oxford, U.K. Reticular erythematous mucinosis (REM) is a rare chronic der- A 35-year-old gentleman presented with a 4-year history of a matosis which has a female preponderance, typically occurring swollen right upper eyelid. It had never been painful or ten- in the second to fourth decades. We report the case of a der. He denied any trauma to the area. He was otherwise well 48-year-old woman who developed REM over the site of a and took no medication. He had been prescribed prednisolone mastectomy, 4 weeks after undergoing a right-sided mastec- 15 mg for 6 months, then hydroxychloroquine 200 mg twice tomy and axillary node clearance for breast cancer. She did daily for 3 months without improvement. On examination he not receive radiotherapy. She presented to the outpatient had a 1Æ5 cm plaque on the right upper eyelid with no associ- department with a 14-month history of asymptomatic, persist- ated lymphadenopathy. Full blood count, erythrocyte sedi- ent, erythematous infiltrated papules coalescing into a reticula- mentation rate, C reactive protein, complement, rheumatoid ted pattern over the mastectomy site, with an irregular but factor, antineutrophil cytoplasmic antibody, protein electro- well defined border. She had two biopsies, at presentation and phoresis, immunoglobulins, cryoglobulins, rheumatoid factor, 3 months later, to rule out recurrent breast cancer. Given the antinuclear antibodies, extractable nuclear antigens, VDRL, localized nature of this eruption, patch testing to the standard THPA and angiotensin converting enzyme levels were all nor- series (British Contact Dermatitis Group Series) and samples of mal. Magnetic resonance imaging confirmed no orbital the prosthesis, was performed to exclude an allergic contact involvement; and chest, abdomen and pelvis computed dermatitis. Readings at 48 and 96 hours were negative. Histol- tomography were normal. The lesion was excised by a plastic ogy showed a perivascular and periadnexal lymphocytic infil- surgeon. Subsequent histology revealed a light lymphocytic trate, with vacuolar changes along the dermo-epidermal inflammatory cell infiltrate within the dermis with a perivas- junction. A diagnosis of discoid lupus erythematosus was sug- cular and periadnexal distribution. There was focal colonisa- gested. Direct immunofluorescence on skin biopsy was nega- tion of follicular epithelium but no significant cytological tive. Serology for antinuclear antigens and extractable nuclear atypia. A striking feature was prominent intravascular collec- antigens was negative. Staining with alcian blue however was tions of histiocytes, confirmed by CD68 expression. There was strongly positive for mucin in the dermis, confirming a diag- no evidence of vasculitis, endothelial proliferation or intralu- nosis of REM, along with the other histological findings and minal thrombi. No organisms were identified on histochemis- the clinical history and examination. Two other cases of REM try. Intravascular histiocytosis is a rare, reactive condition, following diagnosis of carcinoma of the breast have been characterized by the proliferation of histiocytes within vascular described (Quimby SR, Perry HO. Plaque-like mucinosis: its or lymphatic lumina. It presents as erythematous patches and relationship to reticular erythematous mucinosis. J Am Acad is a benign phenomenon of uncertain significance and aetiol- Dermatol 1982; 6: 856–61; Aparicio Martinez JC, Marcos ogy. In some cases it has been associated with rheumatoid Sanchez F, Juarez Ucelay F et al. [Erythematous reticular muci- arthritis. It has been suggested that it is a variant of reactive nosis associated with breast cancer][Spanish]. Anales Med Interna angioendotheliomatosis, which is a proliferation of endothelial 1990; 7: 75–6) on review of the literature. This case high- cells leading to occlusion of dermal blood vessels resulting in lights the importance of recognising REM as a cause of an microinfarction, and is regarded as a reaction to a disease pro- eruption over the site of a mastectomy. cess such as vasculitis, antiphospholipid syndrome, tuberculo- sis, chronic myeloid leukaemia and Castleman’s disease (Rieger E, Soyer HP, Leboit PE et al. Reactive angioendothelio- matous or intravascular histiocytosis? An immunohistochemi- cal and ultrastructural study in two cases of intravascular DP-11 histiocytic cell proliferation. Br J Dermatol1999; 140: 497–504). Intravascular glomus tumour: a rare variant of a However, this typically presents in a more generalized distri- common tumour bution. Earlier reports considered intravascular histiocytosis to P. Farrant and P. Coburn be a variant of intravascular lymphomatosis with a histiocytic Worthing and Southlands NHS trust, Worthing, West Sussex, U.K. phenotype, but there is no firm evidence to suggest the histio- A 65-year-old man presented with a 3-year history of a very cytes are neoplastic (O’Grady JT, Shahidullah H, Doherty VR, painful localized area on his left upper arm. Over the last six Al-Nafussi A. Intravascular histiocytosis. Histopathology 1994; 24: months he had experienced shooting pains, which he de- 265–8). This is an unusual case of a single erythematous scribed as ‘like being stabbed’. These would last several sec- plaque with intravascular histiocytosis on histology but with onds and typically he would experience three to four attacks no identified underlying aetiology. per day. His past medical history included rheumatoid arth- ritis, controlled by oral methotrexate, requiring monitoring blood tests. The tourniquet was situated directly over the

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 British Society for Dermatopathology 99 painful location, and it was this that initially drew his atten- made and repeat biopsies from 1999 to 2004 had similar fea- tion to the problem. The skin was entirely normal in appear- tures. On all occasions cytokeratin 20 and 18 stains were posi- ance and no mass could be felt on palpation. However, as the tive and cytokeratin 7 negative. However, histology of a symptoms were very striking, further investigation was war- lesional biopsy in 2005 showed pagetoid changes but also an ranted. An exploratory operation was carried out. At the oper- associated atypical squamous proliferation with varying ation a prominent vein was identified, with a noticeable bulge degrees of dysplasia typical of squamous carcinoma in situ. in the vessel wall. The vein was ligated and excised. A tumour Paget’s cells stained positive for CAM 5.2 but not for 34BE12 in the lumen of the vessel and extending through the vessel while the squamous components stained in a reverse manner. wall was identified, composed of a proliferation of small uni- There was a squamous proliferative lesion that closely form cells with eosinophilic cytoplasm and regular nuclei. The resembled a ‘fibroepithelioma of Pinkus’. The latest vulval appearances were those of an intravascular glomus tumour. lesion in 2006 showed a gradation of enteric metaplasia, Glomus tumours are one of the characteristic painful skin glandular dysplasia, in situ carcinoma and invasive mucinous tumours, with eccrine spiradenoma, , neurilemm- adenocarcinoma. There are only a few case reports of extra- oma, angiolipoma and blue rubber bleb naevus. Typically mammary Paget’s in association with a squamous neoplasia. these tumours are pink or purple and painful on palpation. Our patient was initially diagnosed as Bowen’s disease, due to These are commonly situated on the hands, especially the fin- mucin-devoid Paget’s cells and later developed a malignant gers, but can also be found on the head, neck and penis. An squamous proliferation and an invasive mucinous adenocarci- intravascular glomus tumour was first described in 1991 and noma of the vulva, which showed a gradation of enteric meta- is a very rare variant (Beham A, Fletcher CD. Intravascular glo- plasia, dysplasia, in situ carcinoma and invasive mus tumour: a previously undescribed phenomenon. Virchows adenocarcinoma causing clinical and histopathological diag- Arch A Pathol Anat Histopathol 1991; 418: 175–7; Acebo E, Val- nostic difficulties. The presence of metaplasia sheds light on Bernal JF, Arce F. Giant intravenous glomus tumor. J Cutan one of the possible pathogenetic mechanisms in the pathogen- Pathol 1997; 24: 384–9). Although there are very few case esis of Paget’s disease. reports of this variant, there is no evidence to suggest a more aggressive behaviour or increased risk of metastasis. We pre- sent this case to highlight this rare variant and to draw atten- tion to the possibility of this tumour, even when no clinically visible or palpable lesion is present. DP-13 Histiocytic sarcoma G. Kaushal and R. Ratnavel Stoke Mandeville Hospital, Aylesbury, U.K. DP-12 A 72-year-old woman presented with a 1-year history of an Unusual histological features in a patient with enlarging painless lump overlying her right hip. She gave a perianal Paget’s disease: diagnostic difficulties past medical history of carcinoma of the rectum. The solitary S. Gossain and E. Reweily lesion was a warm, erythematous, indurated mass, 8 x 8 cm, Epsom General Hospital, Surrey, U.K. with no attachment to the underlying fascia. There was no An 83-year-old woman presented with an extensive perianal associated lymphadenopathy or hepatomegaly. The remainder skin lesion in 1989 with perianal pruritus, which was diag- of the examination was normal. A biopsy was performed and nosed as Paget’s disease and treated with excision and grafting. the histology showed the dermis to be infiltrated by a mitoti- Ten years later she represented. On perineal examination she cally active spindle cell tumour with a broad Grenz zone has an extensive area extending approximately 4 cm each side between the tumour and the epidermis. There were sheets of of the anus and extending to the vulva of indurated, erythe- spindle cells with large atypical and pleomorphic nuclei with matous, whitish, sclerotic plaques with pigmentation, erosions occasional nucleoli and apoptotic bodies. The tumour was dif- and scarring. Treatment has included topical lignocaine 5%, fusely infiltrated by lymphocytes. The morphology was differ- Trimovate, suppositories for constipation and an anal dilator ent from the previously excised colonic adenocarcinoma. The as well as three excisions in 1998, 1999 and 2002. She has cells expressed CD45, CD68 and, focally, lysozyme. They did also had 2-yearly colonoscopies (the last one in 2004 was not express CD3, CD30, CD2, CD20, CD21, myeloperoxidase, normal) and annual perianal lesional biopsies. Blood tests, TdT, CD34, CD31, S100, Melan-A, desmin, EMA, CD56, CEA, chest radiography and CT images of her abdomen and pelvis HMB45, MNF116, CD15, LCA or Bcl-2, cytokeratins, CD1a, have all been normal. Histology of the lesional perianal skin tryptase or CD117. It was concluded that the tumour was a in 1997 showed features suggestive of Bowen’s disease. Two histiocytic sarcoma. Histiocytic sarcomas are rare but contro- populations of cells infiltrating the epithelium were identified. versial neoplasms that occur in lymph nodes, skin and the gas- Both stained positive for CAM 5.2. However, one population trointestinal tract. Histiocytic sarcomas have the potential for stained for PASD, Hale’s colloidal iron and mucicarmine with being clinically aggressive, most of them with lymph node the other being negative. A diagnosis of Paget’s disease was involvement. However, a subset of cases presenting with

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 100 British Society for Dermatopathology localized disease have a favourable long-term outcome. The arthritis, systemic lupus erythematosus, autoimmune thyroiditis, diagnosis requires exclusion of more common neoplasms by vasculitis, carcinoma, lymphoma and leukaemia, suggesting an extensive immunophenotypic studies. Vos et al. (Vos JA, underlying immune complex deposition. Histology is as above Abbondanzo SL, Barekman CL et al. Histiocytic sarcoma: a with leucocytoclastic vasculitis in some cases. Histological study of five cases including the histiocyte marker CD163. Mod differential diagnoses are granulomatous drug reactions and Pathol 2005; 18: 693–704) examined five cases of noncutane- interstitial . Reports of rheumatoid ous histiocytic sarcoma that expressed CD163. They proposed papules, rheumatoid neutrophilic dermatosis and Churg– CD163 as a specific histiocytic marker, and therefore a useful Strauss granuloma have similar features and some authors tool in diagnosing histiocytic sarcomas. Hornick et al. (Hornick believe these are all part of a histological spectrum of pali- JL, Jaffe ES, Fletcher CD. Extranodal histiocytic sarcoma: clinic- saded neutrophilic granulomatous dermatitis (Sangueza OP, opathologic analysis of 14 cases of a rare epitheloid malig- Caudell MD, Mengesha YM et al. Palisaded neutrophilic nancy. Am J Surg Pathol 2004; 28: 1133–44) analysed 14 cases granulomatous dermatitis in rheumatoid arthritis. J Am Acad (seven arose from soft tissue) and concluded all their cases Dermatol 2002; 47: 251–7.) Early lesions have a dense neutro- expressed LCA, CD45RO and CD68. They were all negative to philic infiltrate ± leucocytoclastic vasculitis and degenerated CD21, CD35, CD3, CD20, CD34, myeloperoxidase, HMB-45 collagen, while established lesions reveal palisaded granulomas, and keratins. A definitive marker was not diagnostic in their altered collagen and nuclear dust with variable numbers of cases. neutrophils and eosinophils. Advanced lesions additionally have dermal fibrosis. Therapeutic options include topical or oral corticosteroids, nonsteroidal anti-inflammatory drugs, dapsone, hydroxychloroquine and methotrexate. Exacerbation with phototherapy and positive immunofluorescence may indicate a tendency towards a connective tissue disease in our patient. DP-14 Phototherapy exacerbated interstitial granulomatous dermatitis: part of the histological spectrum of palisaded neutrophilic granulomatous dermatitis R. Suchak, M. Singh, J. Jones, S. McBride and DP-15 M. Rustin Post-irradiation Merkel cell carcinoma Royal Free Hampstead NHS Trust, London, U.K. A. Gulati, R. Verdolini, C. Proby, C. Harwood, An 18-year-old woman gave a 5-year history of a persistent G. Moir, S. Nicholson and R. Cerio rash on her limbs, trunk and face. There was no arthralgia. Skin Tumour Centre, Bart’s and the Royal London NHS Trust, London, U.K. Examination revealed scattered urticated papules and annular Merkel cell carcinoma (MCC) is a rare skin cancer of neuro- plaques on the arms and face, as well as some lichenoid endocrine origin and considered one of the most dangerous papules on the abdomen. Inflammatory markers, serum ACE forms of skin cancer. With an annual incidence of only 0Æ23 and a chest X-ray were normal and autoantibodies (ANA, per 100 000 in white populations (Miller RW, Rabkin CS. ENA, dsDNA, ANCA, RhF) were negative. Initial histology was Merkel cell carcinoma and melanoma: etiological similarities consistent with the clinical impression of granuloma annulare and differences. Cancer Epidemiol Biomarkers Prev 1999; 8: 153–8) on the limbs and lichen planus on the abdomen. Direct im- and a mortality of 40–70% over 5 years, early diagnosis is munofluorescence (IMF) on a biopsy from the arm showed crucial. It occurs in elderly patients on sun-exposed sites, granular basement membrane staining with IgM, and from the especially head and neck regions and presents typically as abdomen a ragged fibrin band. Following one treatment with asymptomatic telangiectatic violaceous nodules with a differ- bath PUVA, there was an exacerbation of her condition with ential diagnosis including lymphoma, basal cell carcinoma and multiple erythematous annular dermal plaques on her limbs amelanotic melanoma. MCC has been reported in immuno- and face. A further biopsy from her left forearm revealed compromised individuals, such as organ transplant recipients marked superficial dermal oedema with a moderate dermal (Buell JF, Trofe J, Hanaway MJ et al. Immunosuppression and infiltrate of lymphocytes and histiocytes palisaded around foci Merkel cell cancer. Transplant Proc 2002; 34: 1780–1). To date, of necrobiotic collagen. Neutrophils and eosinophils were pre- approximately 50 MCC cases have been reported subsequent sent, and there was evidence of leucocytoclasis, consistent to radiation therapy, with tumour development sometimes with a diagnosis of interstitial granulomatous dermatitis many years after radiotherapy. We would like to present a (IGD). Direct IMF was positive with a granular basement 77-year-old lady who had wide areas of Bowen’s disease membrane zone band with IgM and C3. Topical tacrolimus affecting her scalp treated with radiotherapy. Eight years later 0Æ1% twice a day and dapsone 50 mg daily were commenced she developed multiple scalp nodules, which on histology with some improvement. IGD is the preferred designation of a confirmed MCC. Histology characteristically shows monoton- clinically heterogeneous entity that may present as linear cords ous round cells with scanty cytoplasm in the dermis, vesicular (the ‘rope sign’), papules or plaques. Most patients have nuclei with finely dispersed chromatin and small nucleoli, arthralgia. Reported systemic associations include rheumatoid abundant mitoses and apoptotic bodies which may be mis-

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 British Society for Dermatopathology 101 taken for several ‘blue cell’ tumours, such as small cell lung with topical and oral steroids and subsequently azathioprine, cancer, Ewing sarcoma and neuroblastoma. Diagnosis is con- dapsone and mycophenolate mofetil. Moderate control has firmed and differentials excluded by immunohistochemistry, been maintained over 2 years with mycophenolate mofetil, which shows positivity for epithelial stains, such as cytokeratin prednisolone and topical steroids. Patient 3: a 56-year-old 20 giving a typical perinuclear dot pattern, thyroid transcrip- man, with a background of chronic lymphocytic leukaemia, tion factor and neuroendocrine markers, specifically neurone- presented with swelling on his left arm and discrete boggy specific enolase, chromagranin and synaptophysin. Staging by plaques following possible insect bites, which had the appear- imaging was performed. This case raises a number of interest- ance of cellulitis and were initially treated with antibiotics. ing points regarding the aetiology, clinical and histological Subsequently prednisolone led to full resolution but there diagnosis of this rare aggressive skin cancer. It also highlights were recurrences of similar inflammatory lesions on this the difficulties associated with standardized effective manage- patient’s limbs over the next 3 years. Patient 4: a 35-year-old ment strategies of MCC. Its aggressive nature results in early man presented with blisters on the sole of his right foot, lymphatic and haematogenous spread necessitating adequate inflamed papules on both palms and an area of induration on staging of disease in regional lymph nodes, lungs and liver to his back possibly following insect bites. His right foot became plan an optimal treatment strategy. Nodal disease is associated more cellulitic in appearance and he was started on antibiotics. with a poorer prognosis. Evidence-based treatment options are He was systemically unwell with progressive generalized lacking but as in our case often involve a combination of local erythema and woody induration of his trunk and limbs. Blood resection, regional radiotherapy and chemotherapy, with indices revealed both a neutrophilia and an eosinophilia. Fur- radiotherapy offering considerably improved survival rates as ther deterioration prompted HDU admission. He made a grad- expected from a neuroendocrine tumour. ual recovery following 3 weeks of treatment with high-dose systemic steroids. He has had several milder recurrences of inflammatory lesions over the following 18 months. He remains on a low-maintenance dose of prednisolone and methotrexate. His persistent blood eosinophilia is controlled DP-16 with hydroxycarbamide. Biopsies from patients 1 and 2 Well’s syndrome: a spectrum of presentations showed subepithelial oedema and heavy eosinophilic infiltrates S. Desai, S. Halpern, R. Lindley and J. Cooper with flame figures in the dermis. In addition, biopsies from Medway Associated University Hospital, Kent, U.K. patient 2 showed subepithelial splitting with neutrophilic infil- We present four patients who illustrate varied clinical presen- trates, raising the possibility of bullous pemphigoid. However, tations of dermatoses which have been given the histological direct and indirect immunofluorescence was repeatedly nega- diagnosis of Well’s syndrome or eosinophilic cellulitis. Patient tive. Patient 3 demonstrated flame figures and heavy infiltrates 1: a 68-year-old woman, presented with pruritic, erythema- of small lymphocytes and eosinophils involving the epidermis, tous papules and nodules over the trunk and limbs. She was dermis and subcutis. Biopsies from patient 4 also demonstra- systemically well. She had a spontaneous remission over 2–3 ted a panniculitis with deep infiltrates of eosinophils with weeks following her initial presentation. Patient 2: a 71-year- flame figures. The features of eosinophilic cellulitis/Well’s old woman, presented with an itchy blistering eruption cover- syndrome may represent a reactive pattern triggered by a ing her trunk and limbs, resembling but not typical of, number of aetiological factors. bullous pemphigoid. She was systemically well. She was treated

Posters

DP-17 involvement. There was an associated pyrexia but with no Acute generalized exanthematous pustulosis other systemic upset. Apart from stasis eczema she had had no induced by bisoprolol personal or family history of skin disease. She had experienced S.Y. Ng and J. Wilkinson a troponin-positive event while an inpatient on a general med- Amersham Hospital, Amersham, Buckinghamshire, U.K. ical ward one and a half weeks prior to the onset of this rash A 79-year-old lady was admitted to the dermatology ward for and had been started on several cardioprotective medications management of her leg ulcers following bullous cellulitis. including a statin, bisoprolol, aspirin and clopidogrel. The his- During her admission she developed a generalized, symmetri- tory of preceding drug exposure suggested that the general- cal, erythematous eruption suggestive of a drug rash. Approxi- ized pustular eruption was drug induced. The evolving state mately 2 days later the papules developed into pustules with of the rash and the onset of overt pustules led to the b-blocker multiple pustules affecting her trunk and limbs. Her palms being regarded as the most likely drug. Histology revealed and soles were spared and there was no mucous membrane several subcorneal vesicles or pustules containing predomin-

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 102 British Society for Dermatopathology antly neutrophils, and adjacent epidermal spongiosis with an is in keeping with a number of other related studies. In a oedematous papillary dermis with a diffuse inflammatory infil- retrospective study patients receiving 1,25(OH)2D3 along with trate comprising lymphocytes, macrophages, neutrophils and standard immunosuppression had improved graft survival. eosinophils. Following discontinuation of the b-blocker and There is also animal data showing prevention of chronic allo- weak topical steroid application to the affected regions, the graft rejection with use of vitamin D receptor agonists. There rash rapidly improved with desquamation heralding the onset have been no previous studies examining vitamin D polymor- of complete resolution. Acute generalized exanthematous pus- phisms and SCC or BCC outcome in renal transplant patients. tulosis is an infrequent cutaneous eruption that is most often Any risk for skin cancer would be compounded by the longer provoked by drugs. The most common drugs precipitating this graft survival and is, therefore, a true negative association. condition are antibiotics, particularly the b-lactam antibiotics, but antifungal agents, nonsteroidal anti-inflammatory drugs, analgesics, antiarrhythmics, anticonvulsants and antidepressants have all also been implicated (Belder Junior W, Ferolla AC. Acute generalized exanthematous pustulosis (AGEP). Case DP-19 report. Rev Inst Med Trop Sao Paulo 2005; 47: 171–6). We pro- Toll-like receptor 4, 7 and 8 polymorphisms have no pose that in the absence of a personal or family history of association with skin cancer in renal transplant psoriasis, with spontaneous resolution following withdrawal patients of the offending drug and the presence of eosinophils in the M. Laing, P. Dicker, L. Ho, F. Moloney, D. Shields inflammatory infiltrate and the absence of features histologic- and G. Murphy ally suggestive of psoriasis this patient suffered an acute gener- Department of Dermatology, Beaumont Hospital, Dublin, Ireland alized pustular drug reaction rather than a drug-provoked Renal transplant recipients on immunosuppressive therapies have pustular psoriasis (Spencer JM, Silvers DN, Grossman ME. Pus- a greater than 100-fold increased risk of developing nonmel- tular eruption after drug exposure: is it pustular psoriasis or a anoma skin cancer (NMSC) as early as 5 years post-transplantation pustular drug eruption? Br J Dermatol 1994; 130: 514–19). and up to 250-fold risk up to 20 years post-transplantation. While certain risk factors for skin cancer in renal transplant patients are known, they do not adequately explain why some transplant recipients develop NMSC, which develops early, or behaves in an aggressive fashion. Immunosuppression may be DP-18 the trigger to tumour development in this group. Toll-like Vitamin D receptor polymorphisms have no receptors (TLR) are a family of lipoproteins which recognize association with nonmelanoma skin cancer pathogen-associated molecular patterns. These molecular pat- in renal transplant patients terns are highly conserved among large classes of microbial M. Laing, P. Dicker, F. Moloney, D. Shields and pathogens and help discriminate between self and non-self. G. Murphy On recognizing these pathogens, the TLRs trigger increased Beaumont Hospital, Dublin, Ireland transcription of immune response genes via the downstream Vitamin D has potent antitumour properties. Calcitriol signalling molecule MyD88. Polymorphisms in TLRs have

[1,25(OH)2D3], the hormonal derivative of vitamin D3,isan been associated with increased susceptibility to infection, and antiproliferative and prodifferentiation factor for several cell lower rates of atherosclerosis and cancer. We examined the types, including human squamous cells of the head and neck. frequency of TLR4, TLR7 and TLR8 polymorphisms in 454 renal Several polymorphisms of the vitamin D receptor have been transplant patients with and without skin cancer to determine described, including FokI and ApaI. These restriction fragment whether an association existed. The frequency of TLR4, TLR7 length polymorphisms have been reported to be associated and TLR8 polymorphisms was measured, and the association with the occurrence and outcome of malignant melanoma. of these polymorphisms with the development of NMSC was We have examined the frequency of FokI and ApaI polymor- examined. There was no association between patients with the phisms in 400 renal transplant patients and measured the asso- polymorphism and the development of NMSC. To our ciation with squamous cell carcinoma (SCC) and basal cell knowledge this is the first study to evaluate the association of carcinoma (BCC) and also renal allograft survival. There was TLR polymorphisms and skin cancer in renal transplant no association between patients with the polymorphisms and patients. the development of SCC or BCC. There was, however, signifi- cantly improved graft survival at 3, 5 and 10 years for hetero- zygotes and homozygotes for the T allele (P =0Æ03) of Fok1 vitamin D polymorphism. The FokI mutation is a single nuc- leotide polymorphism that leads to a longer protein being translated (425 amino acids), which has decreased activity. This is the first time that the FokI polymorphism has been associated with improved renal allograft survival. The finding

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DP-20 DP-21 Why has melanoma mortality remained unchanged A congenital scalp lesion consisting of numerous when incidence is increasing: success, Meissner’s corpuscles overtreatment and/or misdiagnosis? M. Barkham, R. Brown and C. Moss N.J. Levell, D. Greenberg* and C. Beattie* Birmingham Children’s Hospital, Birmingham, U.K. Norfolk and Norwich University Hospital, Norwich, U.K. and *Eastern Can- We present a case in which an unusual congenital scalp lesion cer Registration and Information Centre (ECRIC), Cambridge, U.K. is predominantly composed of numerous Meissner’s corpus- The aim of this study was to examine if melanoma incidence cles. This could represent a secondary phenomenon in diffuse and mortality in the Eastern Region of the U.K. changed from neurofibroma, or a novel hamartoma. Our patient is a 2-year- 1991 to 2004. Annual data collected by the Regional Cancer old white boy referred for a congenital scalp lesion, which Registry (ECRIC) from histopathology returns were analysed appears asymptomatic. He was delivered at full term by Cae- for each year from 1991 to 2004. Age-standardized incidence sarean section because of breech presentation. His develop- (for stages 1–4 melanoma) and mortality rates were calculated ment is normal and general health is good. On examination with 95% confidence interval (CI). The Table shows data from there is a large, pink lesion with a cobblestone surface over years 1991, 1996, 2000 and 2004. These and other annual the right posterior scalp. There is associated alopecia, with results show a progressive increase in melanoma incidence profuse growth of hair surrounding the lesion (the ‘hair collar over the study period. By contrast melanoma mortality sign’). The lesion itself is soft and freely mobile over the peri- showed no significant change between 1991 and 2004. Analy- osteum. His skin is otherwise normal. Neither he nor his par- sis of the incidence of melanoma by stage at presentation ents have features of type 1 neurofibromatosis (NF1). showed that the marked progressive increase in incidence over Sensation over the lesion is difficult to assess formally at his this period was due to an increase in diagnosis of stage 1 age, but appears grossly normal. Plain X-rays confirmed the melanoma (Breslow thickness less than 2 mm or less than 1 skull is intact, and magnetic resonance imaging head scan mm with ulceration). There are three possible explanations for showed subcutaneous thickening at the site of the lesion but the increase in incidence in stage 1 melanoma with no change no intracranial abnormality. A diagnostic biopsy and then in mortality nor change in incidence of more advanced mela- first-stage excision have been performed thus far. The speci- noma. There may have been a progressive increase in mela- mens show an ill-defined lesion consisting of numerous noma incidence which by coincidence exactly matched an Meissner’s corpuscles throughout the dermis, extending into increase in early diagnosis to give no effect on mortality or in- the subcutis where they are present either singly or in small cidence of more advanced disease. Secondly, there may have clusters. Some scattered nerve trunks are also seen within the been a change in histopathological diagnostic criteria so that dermis. A small nodule of spindle cells is seen in the upper benign lesions, previously diagnosed as dysplastic naevi were dermis but elsewhere the corpuscles are not associated with increasingly diagnosed as stage 1 melanoma. This could have any spindle cell stroma. Immunohistochemistry: S100 high- been due to changing medical practice by pathologists due to lights the corpuscles and small nerve trunks, but not the spin- increased training or defensive practice. Thirdly, many stage 1 dle cells; glial fibrillary acid protein, neurofilament, melanomas may be a different, relatively benign disease, synaptophysin and neuronal nuclei stains are all negative. which did not necessarily progress or increase mortality when Meissner’s corpuscles are cutaneous mechanoreceptors fre- pigmented lesion removal was less enthusiastically pursued in quently found in glabrous skin, but they can occur at other earlier years. These three explanations are not mutually exclu- sites. They may represent a secondary phenomenon in diffuse sive so one, two or three of these factors may be present. If neurofibromas (Megahed M. Histopathological variants of neu- the unchanged mortality reflects successful efforts by skin can- rofibroma. A study of 114 lesions. Am J Dermatopathol 1994; 16: cer teams to hold back the tide of an aggressive melanoma 486–95); however, no background neurofibroma has been epidemic then these teams can be congratulated. However, if demonstrated in this case. The alternative of a hamartomatous these figures represent overtreatment or misdiagnosis of neural lesion consisting of numerous Meissner’s corpuscles has benign disease we should be critically examining how we tar- not, to our knowledge, been described previously. Our patient get our efforts and resources. clearly has a very unusual congenital lesion. The aim of serial excision is to remove the lesion fully and optimize the even- Table Age-standardized incidence rates per 100 000 and mortality per tual cosmetic outcome. He will require further procedures to 100 000 of malignant melanoma (± 95% CI) achieve this. It is possible that these further specimens may 1991 1996 2000 2004 help establish a more definitive diagnosis. Clinical observation as he matures may also reveal diagnostic clues not currently Incidence 8Æ30 ± 0Æ78 9Æ17 ± 0Æ80 11Æ17 ± 0Æ86 11Æ82 ± 0Æ88 Mortality 2Æ17 ± 0Æ39 2Æ43 ± 0Æ41 2Æ84 ± 0Æ43 2Æ64 ± 0Æ41 apparent, although only a minority of cases of diffuse neurofi- broma are associated with NF1.

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DP-22 DP-23 Recurrent follicular toxic pustuloderma associated Sweet’s syndrome associated with exacerbation of with an undifferentiated connective tissue disease Crohn’s disease – a report of two cases J. Goulding, M. Singh, E. Seaton, V. Swale, E. Wray, C. Devereux, S. McGleenon, J. Collins, S. McBride, M. Rustin and C. Orteu M. Walsh, O. Dolan and A. Bingham Royal Free Hospital, London, U.K. Royal Victoria Hospital, Belfast, U.K. There are multiple cutaneous manifestations of lupus erythe- Sweet’s syndrome, or acute neutrophilic dermatosis, is charac- matosus (LE) and connective tissue diseases but sheeted pustu- terized by fever, peripheral neutrophilic leucocytosis and the lation in the absence of acneiform lesions appears to be acute onset of painful erythematous papules, plaques or nod- exceedingly rare. A 19-year-old black woman presented to our ules. Histology classically shows a dense neutrophilic infiltrate department with a florid generalized exfoliative eruption asso- without evidence of primary vasculitis. It is reported in associ- ciated with profound hypotension refractory to aggressive ation with neoplastic and inflammatory conditions but rarely intravenous fluid therapy. She was admitted to ICU for inva- associated with Crohn’s disease. We report the rare association sive haemodynamic monitoring. She improved slowly with of Sweet’s syndrome occurring acutely in two adults during supportive care and was discharged 10 days later. Over the an exacerbation of Crohn’s disease; one patient had received following 18 months she complained of recurrent painful vul- biological intervention with infliximab. The first patient is val and perineal ulceration, intermittent pustular eruptions, 35-year-old man with a history of severe Crohn’s disease since and scaling affecting the external auditory meatus and scalp. the age of 3 years. Five weeks following his third infliximab She was found to have positive titres for antinuclear antigen infusion he presented acutely with a widespread pustular (ANA) (> 1 : 1000, fine speckled), Ro and La antibodies, and eruption associated with persistent pyrexia, raised inflamma- rheumatoid factor. Double-stranded DNA antibodies and com- tory markers in conjunction with a peripheral neutrophilia plement levels were normal. Treatment with hydroxychloro- and increased bowel frequency. Despite high-dose intravenous quine led to marked improvement, but this drug was stopped antibiotic and aciclovir therapy, clinical deterioration was by the patient after 3 months. Shortly after this she was admit- noted. Blood, urine, stool cultures and skin swabs were all ted with a florid rash comprising multiple annular plaques negative on bacteriology and virology analysis. Computed studded with (sterile) pustules, widespread erythema and tomography scan of the abdomen excluded intra-abdominal striking facial oedema. Admission to ICU was again required sepsis. Diagnostic punch biopsy revealed marked subepidermal because of hypovolaemic shock, and she settled only after and dermal oedema with a marked neutrophilic infiltrate con- pulsed intravenous methylprednisolone. She is currently sistent with a diagnosis of Sweet’s syndrome. A dramatic clin- asymptomatic on a combination of oral prednisolone, myco- ical improvement was seen in 48 h following initiation of phenolate mofetil and hydroxychloroquine. The common high-dose oral prednisolone. The second patient is a 68-year- features on haematoxylin and eosin-stained sections of old woman with a 5-year history of Crohn’s disease. She was multiple biopsy specimens were subcorneal, intracorneal and admitted to a surgical ward with persistent pyrexia, increased follicular pustules, and a pronounced neutrophilic dermal bowel frequency and abdominal pain associated with raised infiltrate. Direct immunofluorescence studies demonstrated inflammatory markers and peripheral neutrophilia. An initial bright ANA staining with IgG throughout the epidermis, but diagnosis of intra-abdominal sepsis was made; however, inves- no lupus band. This case escapes formal diagnostic categoriza- tigations were contrary to this and deterioration was noted tion, but has many features in keeping with amicrobial pustu- despite intravenous antibiotic therapy. On day 12 of her losis of the folds. This is a recurrent, aseptic pustuloderma admission she developed multiple purple ulcerated nodules on involving mainly the skin folds, seen exclusively in young her shins in addition to numerous bullous lesions on her arms females, and most frequently occurs in patients with LE or un- and abdomen. Diagnostic skin biopsy revealed a neutrophilic differentiated connective tissue diseases. Only a handful of infiltrate of the dermis in keeping with Sweet’s syndrome. A cases have been reported in the literature (Lagrange S, Chosi- further dramatic response to high-dose oral steroids was seen dow O, Piette JC et al. A peculiar form of amicrobial pustulosis within 48 h. These two rare cases highlight that Sweet’s syn- of the folds associated with systemic lupus erythematosus and drome can present with severe systemic upset particularly in other autoimmune diseases. Lupus 1997; 6: 514–20; Inui S, association with Crohn’s disease. The varying morphology can Azukizawa H, Asada H, Itami S. Amicrobial pustulosis with cause diagnostic difficulty for the nondermatologist. Recogni- antinuclear antibodies and rheumatoid factor. Br J Dermatol tion is important as it can alleviate concerns regarding the use 2006; 154: 568–9). Our case appears to be the first in which of steroids in the presence of constitutional upset and allow recurrent episodes of life-threatening extensive cutaneous prompt treatment. involvement have been reported.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 British Society for Dermatopathology 105

DP-24 Consequently, a diagnosis of a proliferating pilomatrixoma Proliferating pilomatrixoma – an unusual clinical and was made. Pilomatrixomas are benign tumours of the hair histological variant mimicking nodular malignant matrix. Commonly they present as a solitary, small red firm melanoma in the elderly papule situated on the head or neck in children. They are only V.H. Smith, S.E. Coupland and R.A.G. Parslew rarely seen in adults when the history may be short and Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, growth rapid (Swerlick RA, Cooper PH, Mackel SE. Rapid U.K. enlargement of pilomatrixoma. J Am Acad Dermatol 1982; 7: 54–6). A 91-year-old lady presented with a 4-week history of a rap- Pilomatrix carcinoma is the rare malignant variant with an idly enlarging nodule on the antitragus region of the right aggressive nature and potential to metastasize that must be dis- ear. Examination showed a 15 · 10 mm nodule of striking tinguished. These present as expanding nodules usually in the clinical appearance. There were focal areas of dark black and elderly patient. They are difficult to distinguish clinically from chalky white pigmentation and surface telangiectasia. There proliferating pilomatrixoma. Malignancy is usually based on a was no cervical lymphadenopathy. The lesion, along with very large predominant basaloid component, cytological aty- some underlying cartilage, was urgently excised to exclude a pia, locally aggressive behaviour with extensive infiltration nodular malignant melanoma. Histological examination and high mitotic activity. Extensive tumour necrosis is also revealed a normal epidermis, with a well-demarcated tumour suggestive of malignancy. Rarely such tumours demonstrate in the dermis consisting of a rim of basophilic cells with scant vascular or lymphatic invasion. Due to such cases having been cytoplasm and some mitotic frequency. The basophilic cells reported to metastasize to the lungs, bone and lymphatics, phased into an intermediate zone of cells in which the nuclei wide local excision and regular follow-up is recommended. assumed a washed-out appearance and the cytoplasm was uni- Our case showed none of these features. This case represents formly eosinophilic (ghost cells). Dystrophic calcification was an unusual clinical presentation of proliferating pilomatrix- also present in this zone. Focally, some of the basaloid cells oma. The clinical appearance highlights an important differen- formed small buds, which extended from the main tumour tial diagnosis of nodular malignant melanoma in the elderly. into the surrounding tissue eliciting a desmoplastic reaction.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 94–105 British Society for Dermatological Surgery: Summaries of Papers

DS-1 DS-2 Dermatoscope use and disinfection in the Impact of introducing dermatoscopy into a teaching dermatology department of a university teaching hospital skin tumour clinic on melanoma diagnosis hospital over a 4-year period: retrospective analysis K. Chittari, J.M. Orendi and B.B. Tan A. Palmer, J. Bowling and K. Hollowood Dermatology and Microbiology Departments, University Hospital of Stafford- Oxford Radcliffe Hospitals NHS Trust, Oxford, U.K. shire, Stoke-on-Trent, U.K. Melanoma incidence rates are increasing. Dermatoscopy has Dermatoscopes are instruments used by dermatologists to been reported to increase the diagnostic accuracy of melanoma improve direct visualization of structures underneath the stra- and melanoma in situ. Dermatoscopy was introduced as a diag- tum corneum. They have been shown to increase diagnostic nostic technique to our skin tumour clinic in October 2004 accuracy for pigmented lesions. As dermatoscopes are often with the expectation of improving the diagnostic accuracy of applied directly to cutaneous and mucocutaneous surfaces on melanoma detection. The aim of this study was to determine multiple patients in sequential fashion, like stethoscopes what effect the introduction of dermatoscopy has had on the (Breathnach AS, Jenkins DR, Pedler SJ. Stethoscopes as possible incidence of melanoma and melanoma in situ diagnosis and vectors of infection by staphylococci. BMJ 1992; 305: 1573–4), the average Breslow thickness in the 2 years following intro- they are not cleaned between patients. One might consider der- duction of dermatoscopy compared with the 2 years preceding matoscopes to be a possible source of transmission of infection. introduction of dermatoscopy. A retrospective study was car- As there is no existing disinfection policy specific for dermato- ried out of patients who attended the Department of Derma- scopes, we carried out a study to identify the risk of transmis- tology’s nonselective skin tumour clinic between October sion of potentially pathogenic bacteria through dermatoscope 2002 and October 2006. All patients diagnosed with mela- use and to identify the best method to reduce the above said noma during this 4-year period were included in the study. risk. Over a period of 4 weeks, we looked at the existing practice Data were obtained from the histopathology department and of dermatoscope use using three different immersion fluids included: histopathological subtype of melanoma, Breslow (Aqua gel, liquid paraffin and rinse-free hand alcohol gel). thickness, Clark level, site of lesion, age and sex of patient. Bacterial cultures were taken from the contact plate lens of der- Between October 2002 and 2006, a total of 653 melanomas matoscopes at the end of each clinic. On the fifth week, a proto- were diagnosed (n = 136, 144, 185 and 188 in years 1, 2, 3 col of disinfection was introduced using 70% sterile isopropyl and 4, respectively). While the number of in situ melanomas alcohol pads to clean dermatoscopes after each patient use. We remained relatively constant (n = 53, 50, 62 and 45 in years examined the impact of bacterial contamination and load after 1, 2, 3 and 4, respectively), there was an increasing number the introduction of this protocol. A total of 66 swabs was taken of invasive melanomas (n = 83, 94, 123 and 143 in years 1, at the end of 66 consecutive dermatology clinical sessions. Fifty- 2, 3 and 4, respectively). This increase in melanoma diagnosis one of them were taken before the introduction of the disinfect- was predominantly seen in the female subgroup with superfi- ant protocol and 15 were taken after. All three dermatoscopes cial spreading malignant melanoma. There was no statistically grew coagulase-negative Staphylococcus species. The highest den- significant reduction in Breslow thickness over this 4-year per- sity of heavy growth of bacteria was found on dermatoscopes iod (P =0Æ2). Although our results suggest dermatoscopy using Aqua gel and liquid paraffin as immersion fluid. Rinse- may have some effect on increasing the detection of mela- free hand alcohol gel had scanty to moderate growth of skin noma during the study period, there was no associated reduc- bacteria. There was no growth of bacteria after the introduction tion in the average Breslow thickness, and no increase in the of the disinfectant protocol from any of the dermatoscopes. A detection of melanoma in situ during this time. These findings variable growth of coagulase-negative Staphylococcus species was suggest that the increase in detection of melanoma cannot be found in this study when the dermatoscopes were not cleaned attributable to the use of dermatoscopy alone. between patients. The introduction of disinfecting dermato- scopes using 70% isopropyl alcohol pads after use on each patient significantly reduced the risk of bacterial contamination regardless of which immersion fluid was used. We recommend cleaning of dermatoscopes after each use with 70% isopropyl alcohol as a simple and effective method to prevent transmission of pathogenic bacteria.

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DS-3 the study was terminated early. The median area of tumour in A randomized study comparing tissue conservation the MMS group was 58Æ1mm2 vs. 45Æ6mm2 in the standard in conventional vs. Mohs’ surgery of basal cell surgery group. The median area of the surgical defects in the carcinoma MMS group was 116Æ6mm2 vs. 187Æ7mm2 in the standard F.M. Muller, R.S. Dawe, H. Moseley and C.J. Fleming surgery group [confidence interval (CI) 61–126, P <0Æ0001]. Ninewells Hospital and Medical School, Dundee, U.K. The median area of healthy tissue (surgical defect minus Basal cell carcinomas (BCCs) are slow growing, locally inva- tumour area) removed in the MMS group was 41 mm2 vs. sive malignant skin tumours. Morbidity associated with BCCs 148Æ67 mm2 in the standard surgery group (CI 84–118, is related to local tissue invasion and destruction. Standard P <0Æ0001). To our knowledge this is the first randomized treatment of small, primary BCCs in the U.K. involves surgical trial showing that MMS is superior to standard surgery with excision with excision margins varying from 3 to 15 mm. regards to tissue conservation. It is likely that this superiority Mohs’ micrographic surgery (MMS) is recognized globally as is greater with infiltrative tumours in high-risk anatomical the gold standard for high-risk BCCs that are recurrent, are sites. morphoeic or are at sites of recognized high risk for recur- rence. The main advantage of MMS over conventional surgery is the chance of complete tumour removal in 99% (Rowe DE, Carroll RJ, Day CL Jr. Long-term recurrence rates in previously untreated (primary) basal cell carcinoma: implications for DS-4 patient follow-up. J Dermatol Surg Oncol 1989; 15: 315–28) for The accuracy of histopathological evaluation of primary and 95% for recurrent tumours. It is also thought, surgical margin control in the resection of based on empirical evidence, to be tissue sparing when com- nonmelanoma skin cancer: frozen-section pared with conventional surgery, but no randomized con- Mohs’ technique vs. formal pathologist review trolled trials exist to support this. One recent randomized R. Barry, R. Ashton,* H. Smith and S. Keohane controlled study, although it was not a primary outcome Portsmouth Dermatology Centre, Portsmouth, Hampshire, U.K.; *Royal measure, commented on defect size and showed that the aver- Hospital Haslar, Gosport, Hampshire, U.K. and Royal Hampshire County age defect size for standard surgically treated lesions that Hospital, Winchester, Hampshire, U.K. required a re-excision was about twice that of the average The dual benefit of Mohs’ micrographic surgery (MMS) relates defect size resulting from multistage MMS when more than to the higher cure rates achieved in the management of pri- one stage was needed, but no firm conclusion regarding tissue marily nonmelanoma skin cancer, allied to the greater preser- conservation was reached (Smeets NW, Krekels GA, Ostertag vation of normal adjacent tissue in cosmetically sensitive facial JU et al. Surgical excision vs Mohs’ micrographic surgery for areas. The efficacy of the Mohs’ technique is dependent upon basal-cell carcinoma of the face: randomised controlled trial. the ability of the surgeon to accurately interpret the frozen Lancet 2004; 36: 1766–72). It has been argued that the most histopathological specimens. The evaluation of margin control important outcome after BCC surgery is the cosmetic and is usually the sole responsibility of the Mohs’ surgeon as, functional deficit following repair. This is dependent on oper- worldwide, most units do not routinely send their samples for ator skills, and irrespective of operator, a smaller defect is eas- formal review by the pathologist. Recently, both the cost- ier to repair. We have therefore completed a randomized effectiveness and necessity of regular usage of MMS has been study investigating tissue conservation in conventional surgery called into question both by health economists and surgical vs. MMS, using final wound defect size as the primary out- colleagues (Essers BA, Dirksen CD, Nieman FH et al. Cost- come measure, to test the hypothesis that MMS spares tissue effectiveness of Mohs micrographic surgery vs surgical exci- more than standard surgery. Patients with a nodular BCC of sion for basal cell carcinoma of the face. Arch Dermatol 2006; less than 1 cm in diameter, at least 1 cm away from the eye 142: 187–94; Griffiths RW, Suvarna SK, Stone J. Basal cell car- or nose were randomly allocated to either MMS or standard cinoma histological clearance margins: an analysis of 1539 surgery. Higher risk tumours were excluded, and were all conventionally excised tumours. Wider still and deeper? J Plast treated with MMS. Wolf and Zitelli suggest 4-mm excision Reconstr Aesthet Surg 2007; 60: 41–7). At our unit, the frozen margins (Wolf DJ, Zitelli JA. Surgical margins for basal cell sections are sent for independent review by a pathologist. We carcinoma. Arch Dermatol 1987; 123: 340–4) to achieve 95% present the data on 500 patients who had Mohs’ surgery per- tumour eradication. Prior to surgery the diameters of the formed by one of three surgeons between June 2005 and tumours were measured using a Vernier calliper and tumour December 2006. The mean age of the group was 66 (range areas calculated using appropriate formulae for approximately 18–95). Four hundred and ninety patients (98%) had a diag- round and elliptical tumours. After surgical removal of the nosis of basal cell carcinoma (BCC) of which 41 (8%) were tumour, digital photographs of the defect including a scale morphoeic and 61 (12%) were recurrent. There were seven were taken. An interim analysis was performed after 30 cases of SCC. Eighty-seven patients (17%) were recipients of patients as outlined in the sample size estimate. Based on pre- solid organ transplants and were receiving immunosuppres- determined rules (the mean defect size of the standard surgery sion at the time of surgery. In 20 cases (0Æ04%), there was group was more than 1Æ5 times larger than the MMS group) discrepancy between the report of the pathologist and the

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 108 British Society for Dermatological Surgery surgeon. The average size of the initial tumour was 30 mm. nonoperating team members often grossly under- or overesti- This subgroup was representative of the group as a whole in mated times required for the procedures. This reflects the need that 19 patients had BCC and there was one SCC. The majority for clearer guidance regarding standardization for timings of were primary BCC with three cases being recurrent lesions surgical procedures. In this audit, most procedures required and one morphoeic. This discrepancy was significant in two less time than requested on the referral form, indicating a patients who required further intervention for residual disease. potential to improve efficiency and service. We have now In the other 18 cases, the discordance was due to annotation designed a new proforma including mean times for proced- differences, notation error and due to the fact that the Mohs’ ures as a guide. In conclusion, to maximize efficiency several surgeon did not routinely review all slides if the sections clo- patient-specific and operator-specific factors, often predictable, sest to the patient were clear. We believe that these data fur- should be taken into account when allocating time for skin ther validate the practice of experienced Mohs’ surgeons surgery. interpreting their own frozen section specimens.

DS-6 DS-5 Time and motion: testing efficiency in the derma- Time taken for dermatological surgery: a prospective tology procedure setting audit J. Fisher, H. Lotery* and C. Henderson* M. Tewary, A. Affleck and S. Varma University of Southampton School of Medicine, Southampton, U.K. and Queen’s Medical Centre, Nottingham, U.K. *Southampton University Hospitals Trust, Southampton, U.K. There is increasing pressure on dermatologists to provide an Time and motion studies are a validated means of determining efficient service with government initiatives like payment by efficiency and identifying sources of delay in a complex work- results and skin cancer treatment targets. We observed that the ing environment. This analytical technique was applied to the time requested for surgical procedures appeared to be variable dermatological surgical services provided by a university hos- in our department. The aim of this audit was to compare the pital department of dermatology. The objectives were to iden- time allocated for procedures against actual times taken to per- tify any sources of low efficiency in dermatological surgical form the procedures. We looked for patterns in times taken service provision and to make recommendations for increasing for standard procedures by specific grades of operator. We efficiency. Three types of surgical session for dermatology aimed to standardize times given for procedures to improve were observed: consultant-led procedure list, nurse-led pro- efficiency of service and to look specifically for patient groups cedure list and dermatology day theatre list. The activities of needing more time. The audit was done prospectively over 3 staff were noted every 2 min throughout the sessions and the months. A stop clock was used to measure the total time in data compiled for processing in Microsoft Excel. The results theatre and the time for procedure for each patient (first inci- show that treatment of dermatology patients in the day theatre sion to final suture). Types of procedures included punch was less efficient and cost-effective than the other settings. biopsies, shave biopsies, curettage and cautery and scalpel Consultant-led procedure lists were the most efficient. Nurse- excisions. Types of repair included linear closure, flaps, grafts led sessions were cost-effective, but less efficient than the con- and second intention healing. A total of 268 patients, 142 sultant-led lists. Paperwork and generic communication were male and 126 female patients, had skin surgery. One hundred identified as major constituents of nonoperating time. We and eighty-four out of 268 (69%) patients were aged over 60 conclude that the most efficient setting for dermatological pro- years. Excision of basal cell carcinoma was the most common cedures appears to be the consultant-led procedure list. Time procedure (248 out of 268). Linear closure was the most fre- might be saved by issuing generic communications in written quent repair performed. Ninety per cent of procedures were format, and through the use of a well-designed proforma to not within the time allocated. Time was overestimated in reduce time spent on paperwork. Our results demonstrate the 118/155 patients (77%) by approximately 12 min and under- advantages of broad, objective appraisal of practice as opposed estimated in 35/155 patients (23%) by approximately 15 to a simple numerical target-driven approach that fails to min. Time spent in theatre was double the time taken to per- address specific aspects of efficiency. form the actual procedure. Almost double the time was required for older patients. One hundred and thirty-nine out of 268 (51%) lesions for surgery were on the face. Facial procedures took about 10 min longer than on other sites. Allocated times for smaller procedures had been overestima- ted. Juniors took longer to do procedures. Contributory factors for prolonged time in theatre included difficult haemostasis, bandaging (5–14 min), patient disabilities like poor mobility, use of wheelchair, deafness or blindness. Interestingly,

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 British Society for Dermatological Surgery 109

DS-7 anticipated included: length of time to full recovery, difficul- Surgical assessment scale to match surgical ties with activities of daily living and the need for dressing procedures to surgical skills for use in skin cancer changes. Sixteen per cent of patients had ‘very bad pain’ dis- management turbing sleep; 35% moderate; 30% mild; and 19% no pain. L. Paul and J. Britton Twenty-two (35%) patients took no analgesia at all (six of Princess Royal Hospital, Hull, U.K. these were experiencing moderate pain). Seventy-nine per cent Surgical procedures must be performed by an appropriately were not taking any analgesia by the 7th day after the trained surgeon. Often, patients requiring dermatological sur- operation. One patient required analgesics up to 6 weeks after gery are assessed by one healthcare professional, but the proced- surgery. Six patients were given antibiotics by their GP. Sev- ure is undertaken on another surgeon’s list. Dermatology enty-six per cent of patients experienced bleeding through the departments now have numerous surgeons with varying levels dressing at the first dressing change only. There were no sig- of ability. A classification system was devised that grades a nificant problems with dressing changes. Forty-six per cent of patient’s lesion to ensure appropriate matching to the surgeon’s patients described altered sensation, usually in the pulp, lateral skills. The aim of the study was to validate the accuracy of the borders of the distal digit or around the proximal nail fold. grading levels. The classification system uses primarily the site The most frequent complaints were of numbness, tingling, or and the size of the lesion, and the type of procedure. Using this pins and needles. In patients surveyed at 9–12 months, altered classification system, lesions were categorized by a consultant sensation was still present in 9 of 32 (28%), although most dermatological surgeon, a dermatology registrar, a dermatology (5 of 9) were still experiencing improvement. Data were ana- specialist nurse, a GP registrar and a general medicine senior lysed using the v2 test to determine whether postoperative house. A 100% concordance rate was achieved between all the paraesthesia was influenced by the type of operation or the healthcare professionals. This classification system enables the presence of moderate or severe postoperative pain. The associ- most appropriate surgeon to be chosen to treat a patient that ation of altered sensation with certain types of operation was s/he may not previously have seen. It can be used by all health- statistically significant (P <0Æ05). Where the nail was replaced care professionals, regardless of surgical experience, who now to provide protection to the nail bed, the old nail remained in assess patients in the dermatology outpatient department. It place for an average of 5 weeks (range 10 days to 16 weeks). ensures that the operating individual is capable of performing When asked how they would advise a friend undergoing a the procedure. It maximizes the skills and resources of more similar operation 46% patients were generally encouraging. experienced surgeons by minimizing their simpler procedures, Others made specific practical comments about planning for which others are capable of doing. In the current financial the recovery period. Analysis of this postoperative question- climate, this classification will help to determine costing of naire has provided more specific information about the conse- these procedures. It is simple to use and provides a method to quences of nail surgery for patients and allows for a better maintain training and governance standards for dermatologists informed discussion at the time of preoperative consent. It when coordinating the management of cutaneous malignancy also highlights the frequency of altered sensation as a conse- in skin cancer networks. quence of nail surgery, a point which is not well elucidated in standard surgical texts.

DS-8 Postoperative questionnaire survey of patients DS-9 undergoing nail surgery The use of topical imiquimod (AldaraÒ) in the D. Shipley and D. de Berker treatment of lentigo maligna of the head and neck Bristol Dermatology Centre, Bristol, U.K. E. Craythorne and C. Lawrence* As part of an ongoing project to improve the accuracy of Kings’s College Hospital, London, U.K. and *Royal Victoria Infirmary, advice given to patients prior to nail surgery, patients who Newcastle upon Tyne, U.K. had undergone surgery were invited to complete a question- Lentigo maligna (LM) typically presents at sun-exposed sites naire. Questionnaires were posted to 96 patients who had in the elderly patients as an asymmetric, slow-growing, irre- undergone any surgical procedure involving the distal digit or gularly pigmented macule with an irregular indented border. nail; 63 (66%) were returned. Thirty-one patients were 2– 4 With changes in sun-exposure behaviour LM is becoming months and 32 were 9–12 months postoperation. Forty-six more prevalent in middle-aged and younger adults. LM pre- of 63 had surgery on a finger, 17 of 63 patients had toe sur- sents a difficult clinical problem and generally there is little gery (14 great toes). Seventy-eight per cent of patients consensus on the optimum form of management. The recom- thought they had completely understood the nature of the sur- mended guidelines for treatment of LM are surgical excision gery at the time of the operation; 63% of patients felt they with excision margins into clinically normal skin of 2–5 mm completely understood limitations to their normal activities in and others have suggested that up to 20% of cases of LM the 2 weeks after the operation. Limitations not completely would require margins greater than this. However, lesions are

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 110 British Society for Dermatological Surgery commonly large and located on cosmetically important head NICE guidance highlights the scarcity of data regarding pri- and neck sites. Excision may result in a poor cosmetic out- mary care skin cancer surgery activity levels. From their audit come and it is difficult to justify this approach when the risk of nine NHS trusts, the proportion of MM biopsied by GPs of malignant transformation is relatively low. We describe our ranged from 1Æ4–13%, compared to 0Æ7–10% for SCC and experience with the use of imiquimod in a group of patients 1Æ3–8Æ8% for BCC. Our findings show that the GP biopsy rates with LM on the head and neck where surgery was not an in our region are within this range for MM (8%), but are option. Eight patients with histologically confirmed recurrent higher for SCC (15%) and BCC (12%). Over the 3 years inclu- or difficult to treat LM of the head and neck were treated. ded in our study, the proportion of MM biopsied in primary Patients were instructed to apply the cream to the pigmented care reduced by 6% compared with BCC and SCC which both area and to a minimum 1-cm margin around the pigmented increased (by 2% and 6%, respectively). The reasons for this area once a day, 5 days per week for 6 weeks. Each patient change may include the implementation of the ‘2-week wait- was then reviewed with clinical photographs and biopsy if ing time standard’ to fast track potential melanomas and the indicated. Six of the seven patients who developed the brisk drive to manage more ‘nonurgent’ skin lesions in primary inflammatory response had clinical resolution of their LM. care. Interestingly, in the 3 years surveyed, the total number Patients with a clinical response were followed up for an aver- of MM and BCC increased by 39% and 27%, respectively, age of 32 months (range 10–57) without clinical recurrence. compared to a reduction of 18% in the number of SCC. Two of the patients failed to develop a brisk inflammatory Although dermatologists are removing the majority of skin reaction; it was these two who did not achieve a clinical reso- cancers in our region, implementation of the NICE guidelines lution of their LM. Interestingly, it was one of these patients should increase this further over the coming years as the pro- who on stopping application of a concurrent barrier cream portion of skin cancers biopsied from primary care reduces. In and given a second course of imiquimod, did have a brisk addition, the number of new cases of skin cancer is projected reaction and clinical resolution of LM. Our study shows that to continue to increase for many years to come. The challenge imiquimod is potentially an excellent nonsurgical treatment will be to ensure that service provision expands in parallel and for LM particularly in those patients who present with large or that standards of care are maintained to improve outcomes for recurrent lesions on the head and neck. Our observations sup- all patients affected. port the concept that an inflammatory response is a prerequis- ite for treatment success with imiquimod.

DS-11 Completeness of excision of malignant melanomas: DS-10 changing rates in primary care and hospital practice What proportion of skin cancers are managed A. Ranasinghe, J. Gass, N. Tompson, E. Rytina* and primarily by dermatologists? The Oxford experience P. Todd T. McPherson, V. Brown, J. Bowling and R. Turner Departments of Dermatology and *Histopathology, Addenbrooke’s Hospital, Department of Dermatology, Churchill Hospital, Oxford, U.K. Cambridge, U.K. The NICE guidance on skin cancer services ‘Improving Out- The incidence of malignant melanoma continues to rise in the comes for People with Skin Tumours’, published in 2006, U.K. and is the major cause of death from skin cancer (NICE confirms the central role of dermatologists in the management Guidelines. CSG Improving Outcomes for People with Skin of all types of skin cancer. One of the aims detailed in the Tumours including Melanoma. The Manual 2006). Survival is guidelines is to reduce the number of skin cancers currently strongly correlated with the Breslow thickness at diagnosis and managed in primary care. They also emphasize the require- the definitive treatment of primary melanoma is currently ment for local data collection to acquire epidemiological infor- complete surgical excision (Roberts DLL, Anstey AV, Barlow mation and to guide service provision. To address these issues RJ, Cox NH et al. U.K. Guidelines for the management of cuta- we have analysed the skin cancer database acquired by our neous melanoma. Br J Dermatol 2002; 146: 7–17). We assessed histopathology department for 6 months (July to December) the rate of incomplete primary excision of malignant mela- of three consecutive years, 2004 to 2006. The numbers of nomas between the years 2001 to 2005 and 2005 to 2006. malignant melanoma (MM) (including lentigo maligna mela- We compared these results between primary care and hospital noma), invasive squamous cell carcinoma (SCC) and basal cell practitioners. Pathology reports from a British hospital were carcinoma (BCC), and the department or GP surgery in which analysed retrospectively. Patients undergoing primary excision the sample originated were noted. In this 18-month period, of melanoma by primary care practitioners and hospital practi- 1894 of 3276 (58%) skin cancers originated from dermatolo- tioners were identified. The completeness of excision was gists. The rest were obtained from plastic surgery (29%), GPs assessed at deep and peripheral margins. Metastatic or recur- (12%) and 1% from other hospital departments (for example rent lesions and diagnostic biopsies were excluded. Six hun- oral surgery, ENT). By histological type 59% of MM samples, dred and eight patients had a primary excision of melanoma 42% of SCCs and 62% of BCCs were from dermatology. The between January 2001 and December 2005, of which 7%

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 British Society for Dermatological Surgery 111 were excised by primary care practitioners and 93% by hospi- or haematoma, colour or texture mismatch, contracture or tal practitioners. The mean age was 60 years, and 53% were hypertrophy. A recent study found graft hypertrophy to be the female. A total of 16% were incomplete excisions. Compared commonest complication of FTSG (Leibovitch I, Huilgol SC, with hospital practitioners, primary care practitioners had a Richards S et al. The Australian Mohs database: short-term significantly higher rate of incomplete excision (14% vs. 36%, recipient-site complications in full-thickness skin grafts. Derma- P =0Æ001). Prior to excision, there was a clinical diagnosis of tol Surg 2006; 32: 1364–8). This can look unattractive to the melanoma in 64% of incompletely excised lesions from hospi- patient and dermatologist and so corrective treatment is often tal practitioners, compared with only 20% of incomplete exci- used. Treatment options include silicone gel sheets, topical sions from primary care practitioners. Following these results, steroids, intralesional steroid injection, dermabrasion and CO2 we then examined the multidisciplinary team records of 110 laser. Gentle manual massage can help in smoothing and soft- patients treated for melanoma over 7 months between August ening a FTSG rendering it more pliable and mobile. The cause 2005 and February 2006. Fifteen per cent of these were per- of this complication is unknown although the site of the formed by primary care practitioners. Compared to hospital abnormal swelling is considered to be dermal rather than epi- practitioners, primary care practitioners had a significantly dermal. It may be a similar process to that of hypertrophic higher rate of incomplete excision (16% vs. 56%). In conclu- scars. Our case is probably an extreme form of graft hypertro- sion we have shown that, in our area, treatment of malignant phy as it settled spontaneously and highlights that active inter- melanomas by primary care practitioners is associated with vention may not be needed. Surgical dermatologists should be higher incidence of incomplete primary excision and uncertain aware of the ‘chewing gum’ complication of a FTSG and that clinical diagnosis. Furthermore, primary care practitioners are conservative management may be all that is necessary. Future now excising more melanomas, with a high percentage of studies comparing different treatment options in the manage- incomplete surgical clearance. As per NICE guidelines, we ment of FTSG hypertrophy would be useful. recommend that all lesions clinically suspected of melanoma should be referred for specialist treatment to an operator with appropriate expertise for the tumour site.

DS-13 A case of DIY Mohs’ surgery using bloodroot obtained from the internet DS-12 A. Affleck and S. Varma* ‘Chewing gum’ hypertrophy complication of a Department of Dermatology, Ninewells Hospital and Medical School, Dundee, full-thickness skin graft on the nose: spontaneous U.K. and *Department of Dermatology, Nottingham University Hospitals resolution over 4 weeks NHS Trust, Nottingham, U.K. A. Affleck and S. Varma* We wish to highlight a potentially serious problem of self- Department of Dermatology, Ninewells Hospital and Medical School, Dundee, treatment of a basal cell carcinoma (BCC) by a patient who U.K. and *Department of Dermatology, Nottingham University Hospitals obtained bloodroot (Sanguinaria canadensis) from an internet site NHS Trust, Nottingham, U.K. (www.cancerx.org/). A number of patients are seeking alter- We bring to attention an uncommon and little reported com- natives to surgery and may be seduced by reports of success plication of a full-thickness skin graft (FTSG) on the nose. We with home treatment using various botanical preparations believe this to be a gross example of graft hypertrophy such including bloodroot which act as escharotic agents. Late recur- that the FTSG resembled a piece of chewing gum stuck on the rences and metastasis following self-treatment have been tip of the nose. Fortunately, this settled quickly and spontane- reported (McDaniel S, Goldman GD. Consequences of using ously. An 80-year-old man underwent Mohs’ micrographic escharotic agents as primary treatment for nonmelanoma skin surgery to a squamous cell carcinoma on the right nasal tip cancer. Arch Dermatol 2002; 138: 1593–6). A 51-year-old man and was clear after two layers. A FTSG was fashioned from the presented with a 2-month history of a 5-mm lesion on the preauricular cheek with a tie over bolster. At 7 days, the FTSG dorsal nose skin. A shave biopsy revealed BCC. He was offered was pink with excellent contour, texture and colour match. Mohs’ micrographic surgery (MMS) but was very upset by the The patient returned unexpectedly after 4 weeks with a thought of aesthetic disturbance to his nose. Following long 10-day history of elevation of the graft. No clot or fluid could discussions and despite being fully informed he decided to be evacuated. The graft continued to thicken and became ele- self-treat with bloodroot. His rationale was that this was the vated, resembling a lump of dried out chewing gum 2 weeks same preparation that was used by Fred Mohs when he later. Removal and replacement of the graft was considered described MMS. He used this on his nose each day for 8 con- but as the patient was due to go on holiday he preferred no secutive days. He described 3 days of agonising pain. After 2 intervention. Gradually the FTSG flattened spontaneously over weeks, a large eschar of fixed tissue separated to leave a 4 weeks with an excellent long-term aesthetic result. FTSGs wound which then took 2 weeks to heal. He initially experi- are often a good choice for defects on the nose. Occasional enced a good response and was anticipating a cure, but then complications include graft failure, infection, acute bleeding applied the bloodroot on two further occasions as he felt the

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 112 British Society for Dermatological Surgery cancer was returning. He has been left with a fair cosmetic suturing experience used the instrument to assist suturing of outcome and remains under long-term review. He has had 35 postexcision skin wounds. The design and use of the two subsequent biopsies to papules that have developed at the instrument is demonstrated (video clip). Questionnaire results scar site which have not shown BCC (12 months review). This of the opinions of operators following its use and a review of patient misinterpreted bloodroot treatment as self-MMS. He the literature of suture needle safety are presented. The tech- failed to appreciate the importance of microscopic control nique required is relatively easy to learn with minimal train- after the central eschar had separated, the technique originally ing. Confidence is gained with repeated use of the instrument. pioneered by Frederic Mohs in the late 1930s using chemical All operators reported the instrument as very successful in fixation in situ by means of a paste containing bloodroot and reducing the risk of needle stick injury and enhancing safety. zinc chloride prior to excision. This version of MMS was Global assessment was reported as excellent overall by three of known as chemosurgery and has now been almost completely the four operators. No clinically significant needle blunting replaced by the Mohs’ fresh-tissue technique which is quicker was noted during its use. The process of learning any new and less painful for the patient. Fortunately, our patient is free technique, differing from previously learned practice, will of tumour thus far and he is now prepared to undergo con- always require a degree of training and feel slightly awkward ventional MMS should he develop a recurrent BCC in the initially. We have shown that the skills required to use this future. This case illustrates another dermatological example of device can be learnt easily by operators at different levels of the power of the internet influencing patient care. Dermatolo- experience. Enclosure of the needle point during knot tying gists should be aware of what patients may be reading and has the advantage of preventing needle stick injury and secur- regulatory agencies should consider reasonable standards for ing the needle in the sterile field. The development of such sites that promote such products. safety devices should be encouraged. Use could be aimed towards trainees or nurses with minimal prior suturing experi- ence so that good skills are learnt early in their career. The authors have no financial interest in this device.

DS-14 Evaluation of a novel ‘needlecatcher’ surgical instrument designed to reduce the incidence of needle stick injuries from suture needles during DS-15 skin suturing Tumescent liposuction: an effective treatment for J.D. Mckenna, S. McGlennon, M. McCallum and juxta-articular Dercum’s disease O. Dolan A.S. Belgi, P.W. Preston, E.J.C. Stewart* and Royal Hospital, Belfast, U.K. R.J. Motley Needle stick injuries are a feared occupational hazard among Welsh Institute of Dermatology, University Hospital of Wales, Cardiff, U.K. surgeons and their assistants, with risk of transmission of and *The Prossor White Dermatology Centre, Leigh, U.K. infectious diseases. Common causes of these injuries are suture A 65-year-old lady with a history of pain around her left knee needles penetrating the surgeon’s nondominant hand during first presented 24 years ago. Pain was severe enough to dis- skin suturing. This risk could be reduced by the use of an turb her night sleep and had a crippling effect on her profes- instrument enclosing the needle point during suture knot sional life as an occupational health assistant. After numerous tying, avoiding the use of forceps or the surgeon’s fingers. visits to a GP, orthopaedic surgeon, physiotherapist, vascular We have been evaluating a novel prototype device not yet surgeon and undergoing various radiological investigations commercially available. It is designed to capture the suture including MRI scan she was finally referred to a dermatologist needle point within an enclosed barrel during knot tying and – 20 years after first developing the symptoms. She was diag- reloading of the needle into the needle driver. The device is nosed with juxta-articular Dercum’s disease and referred to us an adaptation of the Adson style of forceps widely used in for possible treatment with liposuction. On examination, she skin surgery. A hollow barrel containing a central pin is had tender soft swelling on the inferomedial aspect of her left attached to the end of the handle of the forceps. The central knee. The joint itself was not involved. We treated her with pin moves with the opening and closing action of the forceps. tumescent liposuction using 0Æ2% lignocaine with 1 : 2 000 This allows the shaft of a suture needle to be gripped between 000 as local anaesthetic. She had almost instant pain relief and the pin and the inside of the hollow barrel when the needle was very satisfied with the treatment. Recovery was swift and point is placed into the barrel. The needle is held securely uncomplicated and she could walk without pain for the first with the needle point enclosed within the barrel and therefore time after many years. The liposuction procedure lasted only is not capable of causing needle stick injury. Our aim was to 40 min. Three years later she presented to us with juxta-arti- assess how easily the technique used in the operation of this cular Dercum’s disease involving her right knee joint. We trea- instrument could be learned by operators with different levels ted this with tumescent liposuction, again with good effect. of experience and to determine if the operator’s perception of Dercum’s disease or adiposis dolorosa is a rare progressive dis- safety was improved with use. Four operators with varied ease characterized by localized overgrowth of fat associated

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 British Society for Dermatological Surgery 113 with pain. The pain can be out of proportion to physical find- shape of their face than the first three. The combined findings ings and is unresponsive to conventional analgesics. Diagnosis of this and the VEGA study suggest that the increase in TCT of juxta-articular Dercum’s disease is essentially clinical but produced by PLA is dose-dependent. In order to produce an can be easily missed unless sought for. Various treatment obvious improvement, many females with facial volume loss options have been suggested including injection of intraven- will require injection with 100Æ15 g vials of PLA. The costs ous lignocaine, oral mexiletine for pain relief, and surgical of the study were met by the manufacturer but the authors excision. Liposuction using tumescent local anaesthesia is a were not paid and have no financial links with the manufac- quick, safe and effective treatment for Dercum’s disease. It is turer. well tolerated as an outpatient procedure and as the only scars are 1 or 2 small puncture marks it is cosmetically superior to open surgery for this condition.

DS-17 Neofibroplasia misinterpreted as squamous cell carcinoma. A potential histological pitfall of DS-16 fixed-tissue Mohs’ micrographic surgery for Ò A prospective study of poly-L-lactic acid (Sculptra ) squamous cell carcinoma injections in females L. Naysmith, A. Husain, C. Lawrence and J. Langtry G. Fairris and A. Page Royal Victoria Infirmary, Newcastle Upon Tyne, U.K. Private Practice, Winchester, U.K. Mohs’ micrographic surgery (MMS) allows margin control for The injection of eight to ten vials of 0Æ15 g poly-L-lactic acid tumour-negative margins with the potential for tissue conser- (PLA) into the atrophied areas of the cheeks of HIV-infected vation. It is recognized that the histological interpretation of males with lipoatrophy produced a 7Æ2 mm increase in total the more commonly used frozen Mohs’ micrographic sections cutaneous thickness (TCT) and an evident aesthetic improve- of squamous cell carcinoma (SCC) can be difficult. Conse- ment (Valantin M, Aubron-Olivier C, Ghosn J et al. Polylactic quently, fixed-tissue (slow Mohs) has been advocated as an acid implants (New-Fill) to correct lipoatrophy in HIV-infec- alternative to frozen-tissue MMS in order to better distinguish ted patients: results of the open-label study VEGA. AIDS 2003; tumour morphology and staining characteristics from in- 17: 2471–7). Despite its widespread use, to date there have flammatory cells and adnexal tissues. We report a 75-year-old only been anecdotal reports of PLA’s effectiveness in the treat- white man who presented with a 7-week history of an enlar- ment of age-related facial volume loss in females. We sought ging crusted nodule on the left nasal side wall. Examination to address this lack of knowledge. Fifteen female subjects, revealed a 10 x 9 mm discrete centrally crusted nodule which aged 42–59 years, were injected five times over 8 months was clinically consistent with a SCC. A debulking excision was with two vials of 0Æ15 g PLA per treatment. The first three followed by three stages and eight blocks of slow MMS over 6 injection sessions were 4 weeks apart. Then, after assessment days. There was a 4-day interval between stages 1 and 2 and a at 6 months, each subject was given two further treatments. 2-day interval between stages 2 and 3. The histology of the Forty-eight hours before treatment, each vial of PLA was dilu- debulking excision specimen was of a well-differentiated SCC ted with 4 ml of water. Immediately before treatment, 1 ml extending to the margins of the specimen. Stage 1 of the of 2% lignocaine was added. In the lower and mid-face, 0Æ1ml MMS revealed poorly differentiated SCC with perineural inva- ‘sausages’ of PLA were injected under the dermis about 5 mm sion. The second MMS stage showed marked fibroblastic pro- apart using the fanning and cross-hatching techniques liferation and degenerative changes in the muscle with a few and a half-inch 26 gauge needle. In the upper face, 0Æ1ml mitotic figures suggestive of SCC. Somewhat surprisingly, hist- deposits of PLA were injected on periosteum under the orbi- ology of the third MMS stage showed similar changes and cularis oculi or in the temporalis. The PLA was spread evenly prompted immunohistochemistry studies revealing both stages with deep massage. Before treatment, 6 and 12 months later, 2 and 3 negative for Cocktail cytokeratin stain, a specific anti- a 5–12 MHz linear array ultrasound probe with offset body for squamous epithelium. Neofibroplasia is the exuber- (HDI5000, Philips, Philips Medical Systems, Global Informa- ant granulation tissue reaction seen after any surgery. In this tion Center, Eindhoven, Netherlands) was used to measure case the response was overwhelming, possibly in view of the TCT and a Canon EOS 20D camera was used to photograph 4-day interval between the first two stages. Slow Mohs’ tech- the subjects face-on and at a 45 angle. The order of the nique requires more time for tissue fixation with a resultant before and after photographs was randomized and assessed by delay, usually of 1–2 days, between stages of excision. The five members of the lay public while displayed side by side more prolonged delay in this case may be accountable for the on a 19-inch computer monitor. TCT increased by 1Æ3mm neofibroplastic changes, which we have not seen and would (P =0Æ002) after 6 months and 2Æ7 mm after 12 months not have expected to see with frozen-tissue MMS. This case (P <0Æ0001). Only after 12 months, could the observers reli- highlights firstly the potential pitfall of neofibroplasia mimick- ably identify the before photograph. All subjects believed that ing SCC occurring with slow MMS, resulting in unnecessary fourth and fifth injections produced a bigger change in the

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 114 British Society for Dermatological Surgery tissue removal, and secondly emphasizes the value of cytokera- DS-19 tin stains to confirm the presence or absence of SCC. Dermatofibrosarcoma protuberans: an unusual presentation and a challenging case E. Craythorne, A. Robson,* S. Diaz-Cano, J. Salisbury and S. Macfarlane King’s College Hospital, London, U.K. and *St Thomas’ Hospital, London, U.K. DS-18 Congenital dermatofibrosarcoma protuberans of the Dermatofibrosarcoma protuberans (DFSP) is a rare, slow- scalp in a 4-year-old boy excised by Mohs’ growing, infiltrative skin tumour of intermediate malignancy, micrographic surgery with a limited potential for metastasis but a high incidence of L. Naysmith, R. Milner, F. Charlton and J. Langtry local recurrence. It arises in the dermis, subsequently invading Royal Victoria Infirmary, Newcastle Upon Tyne, U.K. deeper tissues. Due to its highly infiltrative growth pattern, Congenital dermatofibrosarcoma protuberans (DFSP) poses a DFSP often extends far beyond the clinical margins; this diagnostic and therapeutic challenge. Not only is this low- accounts for the high incidence of recurrence following stand- grade fibrohistiocytic tumour extremely rare in childhood, but ard surgical excision. Even after wide excision with a 3-cm it may mimic benign-looking lesions such as vascular birth- surgical margin and deep excision to fascia, as is recommen- marks leading to a delay in diagnosis. We report a four and a ded, the reported incidence of recurrence varies from 11 to half year old boy who presented to the paediatricians at 6 20%. Mohs’ micrographic surgery is increasingly being heral- months of age with a small frontal scalp swelling. This was ded for its improved recurrence rate of 0–6%. We report the thought clinically to be a and his parents were reas- rare and interesting case of a 47-year-old Jamaican lady who sured. His family moved to another part of the country and presented with a 4-year history of a large 3 cm, firm nodule he re-presented at the age of 3 years to plastic surgery with on the right shoulder. Surrounding this, there were three the same persistent but enlarged lesion. At this point the clin- smaller hyperpigmented plaques with features suggestive of ical impression was of a sebaceous cyst and it was surgically dermatofibromas. All were separated by at least 5 cm of clinic- excised. Histology, however, showed a pleomorphic spindle ally normal skin. The histology of the initial biopsies was vari- cell proliferation with a storiform architecture occupying the able, with DFSP and dermatofibroma patterns observed in entire dermis and infiltrating into subcutaneous fat. Immuno- different areas. Following multidisciplinary discussion, she histochemistry was positive for CD34, and a diagnosis of underwent wide excision of the central nodule that was felt to incompletely excised DFSP was made. He was referred to our be DFSP, and standard excision of the three satellite lesions. unit when the poorly defined tumour of the scalp measured Similar histological variation was observed in these specimens; 63 x 60 mm. Excision of the tumour by Mohs’ micrographic thus, a diagnosis of DFSP, with admixed features of dermato- surgery (MMS) using the fixed-tissue technique (slow MMS) fibroma, was made. On review of the pathology each of the was carried out with tumour-negative margins after two stages satellite lesions were said to be clear at the margins. Explana- and 20 blocks. He remains free from recurrence at 10 months tions include the possibility of sampling error, skip lesions after surgery. DFSP is rare, with a high recurrence rate but within the tumour, or that the tumours had arisen separately. low potential for metastasis, and usually presents in mid adult The patient had a further wide excision, resulting in a large life on the trunk or limbs. Congenital DFSP is particularly rare defect requiring grafting. Pathological review of the final sam- with a predilection for the trunk and extremities. The initial ple did not contain any residual DFSP. This case raises interest- clinical appearance of congenital DFSP is often a red-blue vas- ing questions regarding diagnosis and management of this cular looking macule or plaque, leading to the misdiagnosis of rare presentation of DFSP. We discuss what value Mohs’ a vascular malformation, before progressing to a more nodular micrographic surgery might have played in our patient, its lesion. Treatment of DFSP is surgical. MMS is favoured over usefulness in showing if these lesions were contiguous and 2–3-cm wide surgical margins as the former offers histological the feasibility of its use in such a large lesion. margin control with likely lower recurrence rates and tissue conservation. The drawback of repeated general anaesthetic exposures for congenital DFSP may limit its use. Congenital DFSP of the scalp is a rare entity but given the locally aggressive nature of these tumours with a high potential for DS-20 recurrence, early diagnosis and adequate surgical excision is The ‘lip switch’ flap for correction of excessive advised. shortening, following excision of squamous cell carcinoma of the lower lip D. Shah, R. Parkin, A. Morris and R. Motley University Hospital of Wales, Welsh Institute of Dermatology, Cardiff, U.K. A 68-year-old lady on long-term immunosuppression for cryoglobulinaemia presented with a rapidly growing squa- mous cell carcinoma on the right side of her chin and lower

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 British Society for Dermatological Surgery 115 lip. This was removed under micrographic control with loss defects. It comes with the reassuring benefit of providing tis- of 40% of the length of the lower lip. The wound healed well sue for a full-thickness graft in the event of unpredictable flap following direct closure, but despite several months of postop- dynamics. erative massage, the subsequent reduction in length of the lower lip created difficulty with mastication, oral continence and oral hygiene. We elected to correct the discrepancy in lip length using a ‘lip-switch’ (or Abbe) flap. The surgical scar on the lower lip was re-incised through the full thickness of the DS-22 lip and a full-thickness triangular piece of upper lip, raised on Bilateral transposition flap: a simple and effective a medial pedicle, which included the labial artery, was rotated method for closure of large defects, especially of the into the defect. The upper lip was closed primarily and the tri- limbs angular flap sutured into the lower lip, thus increasing its S. Dhoat, R. Verdolini, L. Bugatti* and G. Filosa* length. After 3 weeks the pedicle was divided and the remain- Newham General Hospital, London, U.K. and *Ospedale di Jesi, Ancona, ing wounds closed directly. All surgery was performed under Italy local anaesthesia on an outpatient basis. This two-stage pro- Excision of large tumours of the limbs, particularly of the cedure corrected the shortening of the lower lip, reduced the lower legs, can be challenging because of difficulties related to discrepancy in lengths of the upper and lower lip and provi- wound closure. In addition, skin necrosis and the develop- ded a good functional and cosmetic solution to her problem. ment of leg ulcers are significant risks, especially in elderly patients with impaired blood circulation. Large defects may require grafting, with its inherent problems. Flaps can also be used (Tritsch H, Pullmann H. Double-lobed rotation pedicle flap. Hautarzt 1977; 28: 653–7; Keser A, Sensoz O, Mengi AS. DS-21 Double opposing semicircular flap: a modification of opposing Bilateral island pedicle flap repair in a case of lower Z-plasty for closing circular defects. Plast Reconstr Surg 1998; vermilion lip basal cell carcinoma 102: 1001–7), but the methods usually employed to repair L. Naysmith, C. Lawrence and J. Langtry large wounds are not particularly applicable to the limbs. We Royal Victoria Infirmary, Newcastle Upon Tyne, U.K. have developed a simple but effective technique for the clo- The vermilion border of the lower lip is a site more com- sure of large wounds, resulting from the excision of tumours. monly associated with squamous cell carcinoma. Basal cell car- Our method consists of two longitudinal, lobe-shaped flaps cinoma (BCC) at this site is unusual but when present behaves obtained from two opposite sides of the wound. We then in an aggressive fashion. Complete surgical excision and suc- rotate the two flaps around two fulcra placed at the antipodes cessful repair of lower vermilion lip defects can be challen- of the wound by approximately 90. After suture of the two ging. We report the case of a 24-year-old female sun-bed user longitudinal donor areas, the two flaps usually fall into place with a 1-year history of an enlarging crusted lesion on the spontaneously, with no need for pulling or stretching. The central lower lip which had been unsuccessfully treated as her- two flaps are then sutured to the upper and lower borders of pes simplex infection. On examination she had a 1-cm ill- the defect and finally together, with a transversal suture. If defined crusted plaque involving the outer mucosal, vermilion appropriately measured and shaped, this final suture develops and central cutaneous lower lip. A diagnostic biopsy showed a a minimal longitudinal tension, which is ideal for allowing micronodular BCC which was subsequently completely excised the two flaps to adhere to the subcutis. Over the past 12 using formalin fixed horizontal tissue sections (slow Mohs) in months we have treated 13 patients using this technique, 12 two stages and five blocks. Perineural invasion was noted in for either SCC or BCC of the extremities. All had an excellent the first layer, confirming the aggressive nature of the tumour. cosmetic outcome. Healing times were short and neither The final full-thickness defect measured 2 x 1 cm representing necrosis nor infection was encountered. This technique 33% of the vermilion width. Staying within the principles of ensures closure with skin similar in texture, colour and aesthetic reconstruction, the defect was closed with bilateral thickness to the original defect. As such, lobe transposition by island pedicle flaps using tissue of identical colour, thickness rotating opposite twin flaps is now our method of choice for and texture and avoided the unnecessary sacrifice of normal removing large tumours on the extremities. Its advantages tissue and reduction in oral aperture that occurs with a wedge over established flap procedures lie in its simplicity, optimal resection repair. The bilateral island pedicle flap provides an blood supply and low suture tension. excellent functional and cosmetic result for vermilion lip

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 116 British Society for Dermatological Surgery

Posters

DS-23 plus scattered giant cells. There was marked destruction of the Repair of full-thickness defects in the ala using a intima and the artery was partially occluded by fibroblastic rotated island pedicle flap from the cheek proliferation. Within 10 days of steroid treatment his ESR had O. Kerr and R. Motley fallen to 6 mm in the first hour and CRP to < 5 mg L–1, and University Hospital of Wales, Cardiff, U.K. the ulceration started to heal. He remains on prednisolone. Various methods have been described for repairing full-thick- Cutaneous ulceration in the context of giant cell arteritis is ness alar defects – and the choice depends on size, location of extremely rare and suggests extensive vascular involvement the defect on the ala and whether or not there is loss of the since scalp necrosis only occurs when all four supplying arter- alar margin. We describe two patients with large alar defects ies are compromised. It is associated with a mortality rate of (> 50% of the ala) with a full-thickness loss in part and loss 41% and a 67% incidence of irreversible loss of vision – a of the alar rim in one patient. Both defects were adjacent to worse prognosis than in giant cell arteritis without cutaneous the base of the ala (nasolabial fold) and were repaired using a signs. Treatment with high-dose steroids should therefore not skin flap raised from the adjacent cheek on a medially based be delayed. Although a raised ESR and CRP may be helpful in muscular pedicle. The flap was rotated through 180 and making the diagnosis they are not always high, and the preva- sewn into place. In one case the internal mucosal defect was lence of temporal arteritis with a normal ESR has been estima- repaired with a full-thickness graft (harvested from the adja- ted as ranging from 2 to 8Æ7%. Temporal artery biopsy should cent cheek). In the second case, an additional piece of skin be undertaken as soon as possible once treatment has com- distal to the flap was retained, and after de-epithelialization of menced and there is no evidence that this causes further com- the ‘hinge’ turned under – to create an internal lining to the promise of scalp circulation. With adequate corticosteroid nose. Both patients had a good functional and cosmetic out- therapy the prognosis for these scalp lesions is very good and come. We advocate this single-stage flap as a useful option for complete healing is usual. This case highlights the importance some patients with full-thickness alar defects. of dermatologists being aware of giant cell arteritis as a differ- ential diagnosis of scalp ulceration in elderly patients since early appropriate treatment is essential to prevent devastating complications.

DS-24 Scalp ulceration secondary to giant cell arteritis: a diagnosis not to be missed J. Williams and S. Macfarlane DS-25 King’s College Hospital, London, U.K. Improving information given to patients with skin A 73-year-old man was referred by his GP as a 2-week wait cancer: when and how this should be given with a possible SCC. For the past month a painful and crusted B. Balogun-Ojuri and N.J. Levell lesion had been growing slowly on his right scalp. He gave a Norfolk and Norwich University Hospital, Norwich, U.K. history of headaches for the previous 4–6 weeks and felt We aimed to identify the type of information about their con- unwell. Past medical history included hypertension and inter- dition given to target patients (patients referred by GPs with mittent claudication and he was a smoker. On examination he suspicion of possible squamous cell carcinoma or malignant had two ulcerated, crusted lesions with surrounding dusky melanoma). We also wished to identify the most appropriate purple discoloration on the right temporoparietal scalp and a time for patients to receive such information. The audit took further similar lesion on the left upper forehead. There was place at a dermatology outpatient department. One hundred diffuse tenderness on palpation and both temporal arteries and nineteen target patients referred between May and mid- were swollen with no pulse. A clinical diagnosis of temporal July 2006 were audited using a purpose-designed question- arteritis was made. An urgent ESR was only 23 mm in the first naire sent out 4–12 weeks after the diagnosis. Patients were hour, and CRP 12Æ6mgL–1 but because of the clinical find- asked about the type of information received, understanding ings he was treated immediately with high-dose oral predniso- and adequacy of the information and when they felt the infor- lone and omeprazole cover. Skin biopsies were taken the next mation would have been most beneficial. Of the 119 patients day from the scalp lesions, which showed surface ulceration who were sent questionnaires, 68 (57%) responded. Regar- and superficial inflammation with no evidence of malignancy. ding information given to these patients about skin cancer A temporal artery biopsy showed florid giant cell arteritis with prior to attending outpatients, 88% received only verbal infor- prominent fibrinoid necrosis and a chronic inflammatory cell mation by their GP. At the outpatient appointment, 13% said infiltrate in the media, predominantly comprising plasma cells they did not get enough information and 24% were only sat-

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 British Society for Dermatological Surgery 117 isfied to some extent. A majority of all patients, 67%, said the Wycombe, U.K.) was applied for haemostasis. A persistent best time to receive information about their condition was bleeding point was hyfrecated (Conmed, blunt tip, 10 W, when the diagnosis was confirmed, while 13% wanted infor- high) igniting the residual Driclor which burned briefly with mation before diagnosis was confirmed. At diagnosis, 71% a blue flame and self-extinguished. Driclor was introduced as said they had understood completely the information given to a convenient haemostatic without the potential for tattooing them so far about their condition, while 27% understood only the skin. Both of our cases resolved without significant seque- to some extent. Fifty-three per cent did not indicate a need lae beyond distress for patient and doctor alike. However, they for additional information, whereas 32% said they would have highlight the need to exclude flammable products within the preferred written information from the doctor, 9% wanted biopsy room and to constantly re-evaluate the fire risk of contact details for other sources and 3% wanted audio infor- materials introduced to dermatological surgery. mation, as well as information in larger print. This study shows that information needs for skin cancer patients are very variable. A significant number of patients either did not receive enough information or did not understand that given. Twelve to thirteen per cent were particularly unhappy and did DS-27 not recollect verbal information from GPs, were not happy Mohs’ micrographic surgery for recurrent BCC: the with information in outpatients and would have liked infor- Sunderland experience mation before diagnosis. Nearly half the patients would have C. Blasdale and J.A.A. Langtry* liked more information than they received and the majority of Sunderland Royal Hospital, Sunderland, U.K. and *Royal Victoria Infirmary, patients would have liked information to be given at the time Newcastle upon Tyne, U.K. diagnosis was confirmed. Written, audio and larger print in- In the 5-year period, January 2001 to December 2005, 347 formation would have been useful to some, as would direc- Mohs’ micrographic surgical excisions were carried out by a tions to other sources of information. This audit has identified single dermatological surgeon (J.A.A.L.). Fifty-six procedures varying information needs in hospital skin cancer patients and (16%) were performed for clinically recurrent basal cell carci- shown a need for greater flexibility and clarity in explanation. noma (BCC). All lesions were on the head and neck, with the (NICE 2006/09. Guidance to Improve Healthcare Services for Skin Can- commonest sites being the nose and periocular area. The med- cers. Issued 21 February 2006.) No commercial funding was ian time between most recent treatment and presentation with used in this audit. recurrence was 34 months (range 3 months to 20 years). Thirty-seven lesions had been treated previously only once, and 19 lesions treated by two or more previous procedures, up to a maximum of 11. Ten per cent had previously been treated by cryotherapy, 10% by curettage and cautery, 57% by excision and 14% by radiotherapy. A further two cases had DS-26 had a combination of radiotherapy and surgery and one had Fire in the biopsy room been treated by Mohs’ micrographic surgery. The time to pre- G. Wylie and C. Clark sentation of recurrence varied with treatment modality and Glasgow Royal Infirmary, Glasgow, U.K. was shown to be shortest for cryotherapy and longest for Fire is a well-recognized hazard in surgical operating theatres radiotherapy. In 24 cases, histology reports from the time of with the triad of ignition source, fuel and oxidiser. During primary treatment were available. Two of these were pretreat- dermatological surgery oxygen is seldom used and conse- ment biopsies, and 22 excision specimens. Twelve of the exci- quently the risk is reduced. However, ignition can occur with sions were reported to be complete, and 10 incomplete. hyfrecation and hot-wire cautery, most commonly in conjunc- Infiltrative or morphoeic areas were reported in 25%, with tion with alcohol-based cleansing solutions. This risk is now one superficial BCC and all others solid or cystic. No correl- recognized and fires are infrequent but hazards remain from ation was seen when histology at first treatment and at recur- other fuel sources. We report two recent incidents. Case 1: a rence were compared, with all previously infiltrative or woman had curettage for a seborrhoeic wart at her temple morphoeic tumours reported at recurrence as solid BCC, and a using an aqueous aseptic solution (Hibitane, GlaxoSmith similar number of lesions with nonaggressive histology at first Kline, Uxbridge, U.K.). The wound bed was fulgurated using presentation showing infiltrating/morphoeic features at recur- a Conmed hyfrecator (blunt tip, 10 W, high). Scalp hair within rence. Tumour clearance was achieved in 32% after one Mohs’ the surgical field was ignited and fire spread locally before stage, and 34%, 23% and 5% after 2, 3 and 4 stages, respect- being rapidly extinguished. Residual mild skin erythema had ively. Only two patients required more than four stages, and resolved by the next day. It transpires that she had applied one patient declined further treatment before clearance was two different flammable hair styling products just prior to achieved. The median postoperative wound defect was attending for the procedure. Case 2: a patient had a 283 mm2 (range 31–3707); the size of wound was predicted fibroepithelial polyp shaved again using Hibitane. Topical 30% by preoperative lesion size but not by primary treatment type aluminium chloride solution (Driclor; Stiefel, High or time to recurrence.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 118 British Society for Dermatological Surgery

DS-28 DS-29 Quality assurance in the surgical management of Infection rate after dermatosurgical procedures in basal cell carcinomas: an audit of complete excision the dermatology department of a university teaching rates in primary and secondary care hospital M. Griffiths, J. Vella, L. Maxwell, B. Gee and R. Carr K. Chittari and B.B. Tan Warwick Hospital, Warwick, U.K. University Hospital of North Staffordshire, Stoke-on-Trent, U.K. The recent guidance (National Institute of Clinical Excel- This was a prospective observational study to look at the rate of lence. Improving Outcomes for People with Skin Cancers. NICE Febru- surgical wound infection and other factors that influence the ary 2006) states that all patients with skin cancer should be outcome of wound infection such as choice of procedure, ana- offered the same quality of care regardless of where the tomical sites, use of topical antibiotics, and aseptic techniques treatment is carried out and regardless of the grade and used by both operator and assistant during simple dermatosurgi- type of doctor they see. The gold standard for basal cell cal procedures. Procedures involving flaps and graft repair were carcinoma (BCC) management remains surgical excision excluded. Patients were enrolled if they were with clear resection margins. We identified completeness of immunocompetent, not receiving chemotherapy or steroids and excision as a surrogate marker for best practice and under- had not taken antibiotics within the 7 days preceding surgery. took an audit of BCC excision rates in primary and second- The skin was cleaned with chlorhexidine and local anaesthetics ary care. A retrospective audit was undertaken of BCCs were used for all patients. Where indicated, subcutaneous and excised by dermatologists and plastic surgeons (hospital spe- interrupted skin sutures were used. One hundred and seventeen cialists) and general practitioners (GPs) from January 2005 procedures were carried out in 1 month. Seventy-three patients to December 2005, inclusive. The site of lesion, clinical fulfilled enrolment criteria and consented for this study. On fol- diagnosis, histological subtype, peripheral and deep margins low-up, 11 patients were not contactable. Hence, data from 62 were extracted from the pathology reports. A total of 414 patients were analysed. There were 72 procedures carried out in BCC excision biopsies were identified. Of these 217 were this cohort of 62 patients. There were 25 excisions, 20 curet- excised by dermatologists, 156 by the plastic surgeons and tages, 19 incisions, seven punch biopsies and one shave biopsy. 41 by general practitioners. The incomplete excision rate Head and neck constituted 52% of the biopsy sites followed by for the hospital specialists was 6Æ2%, 5Æ6% involving the 18% from the trunk, 13% from the upper limbs, 13% from the peripheral margin and 2Æ0% the deep margin. The incom- lower limbs and 4% from the genitalia. All primary operators plete excision rate for GPs was 24Æ0%, 24Æ0 % involving wore sterile gloves but only 25% of assistants wore sterile gloves. the peripheral margin and 13Æ0% the deep margin. Hospital Seventy wounds (97%) had topical antibiotic polymyxin B sul- specialists excised 86Æ6% of BCCs with more than 1 mm phate application immediately postoperatively before placement margins compared with only 46Æ5% for GPs. Malignancy of dressings. One patient (1%) developed microbiologically pro- was suspected in 95Æ4% of hospital specimens but in only ven Staphylococcus aureus infection on his buttock area where an in- 54% of GP cases. In summary, half of BCCs excised by GPs cisional biopsy took place. He was later treated with oral were not identified as BCCs prior to the removal. Approxi- flucloxacillin. Infection rate after uncomplicated dermatosurgical mately one-quarter of BCCs excised by GPs were incom- procedures in our department is low. Factors such as certain ana- pletely excised. The recent NICE guidance suggests that tomical sites, for example, the genitalia area, and the lack of use low-risk BCCs can be managed in primary care but this of sterile gloves by the assistant do not seem to have contributed audit shows GPs have difficulty in diagnosing BCCs. Inac- to the wound infection. Although often overlooked, topical anti- curate diagnosis results in inadequate management and this biotic agents played an important role in skin surgery. Topical has been shown with melanomas (Khorshid SM, Pinney E, polymyxin B sulphate is widely use in our department. Its bacte- Bishop JA. Melanoma excision by general practitioners in ricidal property provides a safe and effective option in wound north-east Thames region, England. Br J Dermatol 1998; 138: healing (Thornton Spann C, Taylor SC, Weinberg JM. Topical 412–17). GPs must be trained in diagnosis as well as sur- antimicrobial agents in dermatology. Clin Dermatol 2003; 21: 70– gery, regularly attend skin cancer multidisciplinary team 7). Although this is a small study to reach any significant conclu- meetings and be subject to satisfactory audit if they are to sion, it demonstrated that the use of a topical antibiotic may have continue to have a role in the management of skin cancer. a role in reducing postoperative wound infection.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 106–118 British Society for Paediatric Dermatology: Summaries of Papers

PA-1 provided key information which is essential for planning a Results of survey of British Association of study that will best guide clinical decisions regarding treat- Dermatologists (BAD) and British Society for ment. We now plan to submit a proposal to the U.K. Derma- Paediatric Dermatology (BSPD) members regarding tology Clinical Trials Network to develop such a study. treatment of infantile haemangiomas A. Stanway, N. Bassi, H. Williams, S. Varma and J. Ravenscroft Queen’s Medical Centre, Nottingham, U.K. Infantile haemangiomas are a common problem. Current prac- tice in the U.K. is generally to observe uncomplicated haem- PA-2 angiomas because of the likelihood of complete spontaneous Is erythromycin an effective treatment for chronic resolution. Recently, there have been several reports of topical bullous disease of childhood? Results of a national imiquimod for the treatment of infantile haemangiomas with survey of members of the British Society for mixed results, but as yet there has been an absence of ran- Paediatric Dermatology (BSPD) domized controlled clinical trials. In order to test the feasibil- P. Farrant and C. Darley ity of a clinical trial, we conducted a survey to gather Brighton and Sussex Universities Hospitals Trust, Brighton, U.K. information on attitudes amongst dermatologists and paedia- Chronic bullous disease of childhood (CBDC), also known as tricians (with an interest in dermatology) towards usefulness linear immunoglobulin A (IgA) disease (LAD) of childhood, is and design of such a study. Information on number of infan- the most common chronic acquired blistering disorder in tile haemangiomas seen per year, and previous treatment and childhood. It is, however, still a rare diagnosis with an estima- side-effects was also obtained. Postal questionnaires were sent ted annual incidence in the U.K. of 1 per 500 000 children. to all current members of BAD and BSPD in March 2006. The goal of therapy is to control the disease until it spontane- Results were collated by a single investigator. A total of 794 ously remits (usually 2–5 years), without causing any severe questionnaires were sent and 336 were returned completed or long-lasting side-effects. Commonly used first-line treat- (42%). Two hundred and seventy-five (82%) respondents ments include dapsone, sulfapyridine, sulfamethoxypyridazine thought that a randomized controlled trial of imiquimod for and prednisolone, but treatment with these agents can cause infantile haemangiomas would be a useful study to undertake. undesirable side-effects. These include haemolysis, bone mar- The majority of respondents thought that a placebo or no row suppression, skin rashes, weight gain, growth retardation treatment arm would be the most appropriate comparator, n = and diabetes. There are small case series and anecdotal reports 150 (45%). A wide range of other comparators was sugges- of successful treatment of LAD in adults and children with var- ted, with many opting for several treatment arms. One hun- ious antibiotics including the macrolide antibiotic erythromy- dred and forty-seven (44%) respondents said they would be cin. It has been used successfully in bullous pemphigoid and interested in taking part in such a study. Two hundred and in one adult and one child with LAD (Cooper SM, Powell J, fifty-one (75%) of those responding saw at least one child per Wojnarowska F. Linear IgA disease: successful treatment with year with infantile haemangioma, and 11% saw > 10 children erythromycin. Clin Exp Dermatol 2002; 27: 677–9). In this survey per year. Respondents (n = 336) reported a wide range of we were interested in gaining wider experience of erythromy- treatments currently in use including observation only (71%), cin in the treatment of CBDC. An initial questionnaire was laser (61%), systemic corticosteroids (31%), topical cortico- sent via email to all members of the BSPD. A more detailed steroids (20%), intralesional corticosteroids (10%), surgery follow-up questionnaire was sent to those with experience of (10%), interferon (5%), imiquimod (4%), vincristine and cryo- erythromycin in this condition. Sixty-five members responded therapy (both < 1%). Side-effects were seen in all treatment to the initial questionnaire. Over the preceding two years 40 groups: several side-effects were treatment specific. Many free children had been diagnosed with CBDC in the U.K. Twelve text, useful comments were received – most concerned the members had experience of erythromycin in CBDC (with 13 type of haemangioma to be treated and the ethics of conduct- children treated). Erythromycin had been used in five children ing such a trial. Imiquimod is increasingly being reported as a as a single first-line oral treatment. In three of these cases the possible treatment for infantile haemangiomas. Our survey initial improvement was graded as either good or complete suggests that most BAD and BSPD members feel a randomized resolution. This result was only sustained in one child, with controlled clinical trial would be helpful and many are willing the other two relapsing between 4 and 12 weeks. In the to help recruit patients into such a study. This survey has remaining eight children erythromycin was used either in

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 119 120 British Society for Paediatric Dermatology combination with other oral agents or after traditional treat- more flexible service as well as better training opportunities. ments had failed. In six of these children erythromycin was These are the first such data to be published in the field of noted to cause some improvement (5) or good improvement paediatric dermatology. We believe this novel information will (1) and this was sustained in five out of six. It was completely guide future development of a quality dermatology service for unsuccessful in the remaining two children. These results sug- children and their parents. gest that erythromycin is unlikely to produce sustained improvement in CBDC when used as a first-line single agent. It may confer some benefit when used in combination with other systemic treatments, but it is difficult to interpret effi- cacy in this setting. PA-4 Pityriasis lichenoides: the young and the old S. Wahie, E. Hiscutt,* S. Natarajan* and A. Taylor Royal Victoria Infirmary, Newcastle upon Tyne, U.K. and *Sunderland Royal Hospital, Sunderland, U.K. PA-3 Pityriasis lichenoides (PL) is a skin disease of unknown aetiol- Paediatric dermatology in a district general hospital: ogy that can affect both children and adults. It is said to run a evolution of a service over 10 years more benign course in children than in adults, with a frequent M. Kalavala, R. Goodwin and C. Mills tendency to self-resolution (Romani J, Puig L, Fernandez-Figu- Royal Gwent Hospital, Newport, Gwent, U.K. eras MT et al. Pityriasis lichenoides in children: clinicopatho- All aspects of children’s healthcare should be built around the logic review of 22 patients. Pediatr Dermatol 1998; 15: 1–6). child and family, seeing services through their eyes, breaking However, to our knowledge, there have been no published down traditional boundaries and delivering more flexible and studies comparing PL in children and adults. A retrospective responsive care (National Service Framework for Children. review of records from two regional centres was undertaken www.dh.gov.uk/). This study is to assess the evolution of to investigate the clinicopathological features, overall efficacy paediatric dermatology services in Gwent over a 10-year per- of treatments and disease outcomes in children and adults iod along the principles set out in the national service frame- diagnosed with PL between 1998 and 2006. Data were collec- work for children. We started our paediatric dermatology ted on: age, sex, comorbidities, drug history, number of services in 1995 with once a week clinic conducted in the lesions, lesional morphology and distribution, symptoms, paediatric outpatient department. This gives us the benefit of a histopathological features, treatment modalities (and child-friendly environment and access to the paediatric net- response), overall follow-up, length of remission and whether work including a consultant paediatrician, clinical psycholo- malignant transformation occurred. We recorded 25 children gist, dietician and phlebotomist. In 1995, the clinic was run (median age 8 years, range 2–18) and 32 adults (median age by the consultant only. In 2005, the team comprised consult- 40, range 20–65) with PL. All children and adults had more ant, specialist registrar and 1Æ5 specialist nurses. In addition, than 20 scaly, papular lesions. In addition, ulceration and the specialist nurses provide consultations in the day unit, necrosis were present in six children (24%) and eight adults home, school, and by telephone. Both children and parents (25%). Dyspigmentation was significantly more common in liked the flexibility and rapid access to the service. In 1995, children (n = 18, 72%) compared with adults (n = 6, 19%) 244 new patients were seen compared to 409 in 2005. The (P <0Æ001). Children had significantly greater lesional body proportion of infants referred to clinic went up from 11% in involvement than adults. Lesions on the legs and trunk were 1995 to 17% in 2005. In both years about 20% of patients present in 23 children (92%) compared with 19 adults (59%) were teenagers. The three commonest conditions seen in both (P <0Æ01). Facial involvement was observed more commonly years were eczema, naevi and viral warts. The percentage of in children (n = 10, 40%) compared with adults (n =8, patients with eczema seen went up from 20% in 1995 to 30% 25%), although the difference was not statistically significant. in 2005. However, the proportion of naevi dropped from There were no significant differences in the histopathological 21% in 1995 to 15% in 2005. This was surprising, given the features on lesional skin biopsies between each group. Treat- increased awareness about naevi in the general population. In ments included topical corticosteroids which were used by 16 1995, 136 follow-up patients were seen in 211 clinical con- children (64%) and 18 adults (56%) but only half in each sultations. This went up to 407 patients and 715 consultations group found these effective. Eight children (32%) were treated in 2005. Eczema was the most common condition followed with erythromycin, with only two (25%) clearing, but one of up (385/715 consultations in 2005 compared to 109/211 these subsequently relapsed. In contrast, four adults (13%) consultations in 1995). In 2005, more than 85% of eczema received antibiotics with three (75%) clearing and none relap- follow-ups were done by specialist nurses in clinic, home and sing thereafter. UVB phototherapy was used in eight children school with constant access to telephone advice. This freed up (32%) with seven (88%) completely or almost clearing, but the consultant’s time for training the registrar and to manage four (57%) subsequently relapsed within 3 months of cessa- more complex cases. Evolution of our service clearly demon- tion. In contrast, 14 adults (64%) received phototherapy; 10 strates that working in a dedicated team results in better and

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(71%) completely cleared and only two of these (20%) subse- PA-6 quently relapsed. Follow-up in children was for a median of The treatment of viral warts with cidofovir 1%: our 20 months (range 6–30) and 16 months for adults (range experience of seven paediatric cases 2–30). Only five children (20%) went into complete remis- S. Field, A.D. Irvine* and B. Kirby sion compared with 20 adults (78%) (P <0Æ001). There were Department of Dermatology, Adelaide and Meath Hospital Incorporating no significant associations identified between other variables National Children’s Hospital, Dublin, Ireland and *Department of Dermatol- and disease course. To our knowledge, this is the first study to ogy, Our Lady’s Hospital for Sick Children, Dublin, Ireland compare PL in children and adults. Our findings suggest that PL We report a series of seven children treated with cidofovir 1% in children is more likely to run a chronic course than in for persistent, therapy-resistant viral warts. This follows the adults, with greater lesional distribution, more dyspigmentation, successful treatment with cidofovir 1% of a refractory plantar and a poorer response to conventional treatment modalities. verruca in a 9-year-old immunocompromised female patient in 2005 (Tobin AM, Cotter M, Irvine AD, Kirby B. Successful treatment of a refractory verruca in a child with acute lym- phoblastic leukaemia with topical cidofovir. Br J Dermatol 2005; 152: 386–8). Cidofovir is a potent nucleoside analogue antivi- ral agent, which acts as a competitive inhibitor of DNA poly- merase. Cidofovir applied topically in ointment preparation PA-5 has been used for the treatment of resistant human papilloma Filaggrin mutations are a genetic modifier in virus (HPV) (Zabawski EJ Jr, Sands B, Goetz D X-linked ichthyosis: a case report of two brothers with ichthyosis et al. Treatment of verruca vulgaris with topical cidofovir. JAMA A. Waters, H. Liao,* D. Goudie,* F. Smith, 1997; 278: 1236). We carried out a retrospective chart review S. Lewis-Jones and I. McLean* of seven children treated with cidofovir 1% ointment for per- Department of Dermatology, Ninewells Hospital, Dundee, U.K. and sistent, therapy-resistant viral warts. We calculated the cost of *Department of Human Genetics, University of Dundee, Dundee, U.K. cidofovir therapy based on the quantity of ointment used. Mutations that inactivate the steroid sulfatase (STS) gene cause Four of seven children achieved complete resolution of their X-linked ichthyosis (XLI), a form of ichthyosis that affects viral warts in response to topical cidofovir 1% ointment. Min- males and is usually moderate in severity. Recently, loss-of- imal local and no systemic side-effects were reported. All four function mutations in the filaggrin gene (FLG) have been children remained clear at 12-month follow-up. The mean identified as the cause of ichthyosis vulgaris (IV), which is a cost of therapy was €1460 per child. We conclude topical cid- usually milder form of ichthyosis with a semidominant inher- ofovir 1% ointment is a therapeutic option for treatment of itance pattern. Hyperlinearity of the palms is a particular fea- viral warts. We consider the cost was justified in our cases of ture of IV, and in some patients is the only sign of carriage of persistent, therapy-resistant viral warts; however, further stud- a FLG mutation. Variation of the severity in phenotype of the ies are needed to confirm efficacy and safety. genetic ichthyoses is a well-known phenomenon for which the underlying molecular basis is poorly understood. We pre- sent the case of two half-brothers (aged 6 and 9 years) who were born to the same mother following prolonged labours, and who have both suffered with life-long dry skin. The PA-7 younger brother has cardinal features of XLI, with characteris- Audit of the use of topical immunomodulator therapy tic fine dry scaling of the limbs and trunk, and sparing of the in children flexures. The older brother has a much more severe ichthyosis, F. Nayeemuddin and E. Gilmour with widespread ridged hyperkeratosis reminiscent of bullous Tameside General Hospital, Manchester, U.K. ichthyosiform erythroderma. Hyperlinearity of the palms was Our aim was to assess the compliance of topical immunomod- noted in the older brother only, and this prompted us to per- ulator (tacrolimus and pimecrolimus) prescribing to the NICE form FLG mutation testing as part of our investigation. Testing Technology Appraisal guidance issued in August 2004. for the common STS deletion mutation in this family was According to the guidance these treatments should be used as negative. However, both children carried a new STS missense second-line agents in atopic dermatitis (AD) where adequate mutation (T165I), which was not identified in population topical steroid therapy has failed or has serious risk of side- controls. Furthermore, the more severely affected child also effects. We conducted a retrospective audit of 35 children in a carried a heterozygous FLG mutation (R501X), which was district general hospital all of whom had been prescribed a absent from his mildly affected brother. These data show that topical immunomodulator. Of the children prescribed topical interaction between distinct ichthyosis genes, which disrupt immunomodulator, 27 (77%) had AD, six (17%) had vitiligo epidermal differentiation via different pathways, can increase and two (6%) had . The reasons for a trial of phenotypic severity. Due to the high frequency of FLG muta- topical immunomodulator in the AD patients were: failed top- tions in the population, filaggrin should be considered as a ical steroid therapy (19; nine had failed HC 1%, eight possible modifier in other genodermatoses. required more potent topical steroids, two had previously

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 122 British Society for Paediatric Dermatology required oral prednisolone), skin atrophy (1), striae (1), cata- unusual, and particularly remarkable because of their consist- ract (1) and parent’s request (1). In four AD patients the ency in three cases. Possible mechanisms to explain this new rationale for prescribing topical immunomodulator was association include: (i) patients with large CMNs may have an unclear. Treatment was recommended by a consultant derma- abnormality in the level of or sensitivity to a hormone, which tologist in 33 patients (94%) and dermatology nurse practi- accounts for both the development of the CMN and the preco- tioner in two (6%) patients (both licensed indications). cious puberty or (ii) the CMN itself may be producing a hor- Tacrolimus was prescribed off-licence in four AD patients. mone which is interfering with normal pubertal development. There was no evidence of the discussion of off-licence use in It is possible that the discovery of this association will lead us any of the medical records. Treatment tolerance was good in to an improved understanding of the pathogenesis of giant 26 (74%), poor in two (6%) and unclear in seven (20%). CMNs. Therapy was reported successful in 18 of the 35 patients (51%, all AD patients), failed in 13 patients (37% – five AD, six vitiligo and two alopecia areata) and response unclear in four patients (12%). Adverse effects were reported in six (17%) patients, which included eczema herpeticum, recurrent PA-9 bullous impetigo, flare of eczema and folliculitis. Topical Pigmented purpuric dermatosis (PPD): unusual immunomodulators are an effective treatment for AD in chil- presentation in three children dren. Off-licence use of these products in secondary care is S.K. Siddha and R.S. Lever not inappropriate; however, children and their parents should Royal Hospital for Sick Children, Glasgow, U.K. be informed about off-licence use and this should be docu- We report three children who presented with pigmented mented in the medical records. Eighteen of 27 (67%) of chil- purpuric dermatosis (PPD) with an unusual distribution: two dren prescribed topical immunomodulators for AD were had a striking linear, pseudodermatomal distribution (Riordan recorded as gaining better control of their disease at follow- C, Darley C, Markey AC et al. Unilateral linear capillaritis. Clin up. No positive clinical responses were observed in the treat- Exp Dermatol 1992; 17: 182–5) and the third had an unusually ment of vitiligo or alopecia areata. widespread generalized involvement. Patient 1: A 9-year-old girl presented with a 6-month history of a discoid petechial rash on her right thigh, which had gradually spread to involve the right lower leg and right flank. She had had a slight sore throat 4 weeks preceding this, but otherwise had not been PA-8 systemically unwell. Although florid, the rash was showing Congenital melanocytic naevi and early secondary early signs of resolution when she was first seen and had vir- sexual characteristics: a new association tually cleared 18 months later without treatment. Patient 2: V. Kinsler, C. Brain,* P. Hindmarsh* and D. Atherton An 11-year-old boy was referred with a 3-year history of a Great Ormond Street Hospital for Children, Dermatology Department, London, recurrent linear petechial rash on his right arm extending onto U.K. and *Great Ormond Street Hospital for Children, Endocrinology Depart- his trunk. Potent topical steroids produced some improvement ment, London, U.K. but the rash flared on their withdrawal. The condition Large congenital melanocytic naevi (CMNs) are relatively rare, improved after a sunny 2-week holiday in Cyprus in 2006 with an incidence of around 1 in 20 000 live births. A minor- and has remained clear over the following 4 months. Patient ity are known to be associated with central nervous system 3: A 2-month-old girl presented with a 3-week history of an (CNS) abnormalities detectable on magnetic resonance ima- extensive generalized petechial rash, affecting her scalp, face, ging (MRI). To our knowledge there are no reports of endo- trunk and limbs. She was started on oral ascorbic acid and crine abnormalities in patients with CMNs, although it would rutoside (a bioflavonoid) which had been reported to be of be a conceivable complication of intracranial involvement. We benefit (Reinhold U, Seiter S, Ugurel S, Tilgren W. Treatment report three children with congenital melanocytic naevi and of progressive pigmented purpura with oral bioflavonoids and satellite lesions who presented with clinical signs of preco- ascorbic acid: an open pilot study in 3 patients. J Am Acad Der- cious secondary sexual characteristics. All three children have matol 1999; 41: 207–8). The rash improved gradually over the had normal MRI scans of the CNS. Three girls known to have following 5 years whilst on treatment, although this may have large CMNs (all will be > 20 cm in adulthood) with satellite been spontaneous resolution rather than a response to treat- lesions presented at the ages of 8 months, 2 years 10 months, ment. Now at the age of 6 she is virtually clear. The three and 7 years. All three presented with signs of breast develop- patients showed similar features: apart from a possible viral ment, and the eldest one also with pubic hair. Initial investi- infection in Patient 1, there was a lack of any significant pre- gation revealed raised baseline FSH levels (4Æ5, 1Æ7 and 1Æ5IU ceding illness and none had received any preceding medica- L–1, respectively) with normal (undetectable) levels of LH. tion. Routine investigations included a full blood count, urea, Two of the three have had an LHRH stimulation test which electrolytes, liver function tests and coagulation profile and showed a predominantly FSH response. Levels of other pituit- were all within normal limits. Skin biopsy showed features in ary hormones and of oestradiol were normal. These results are keeping with PPD in all the three cases. PPD is usually

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 British Society for Paediatric Dermatology 123 considered a capillaritis of unknown cause and is characterized rare disorder and discuss the possible mechanisms in its path- by diffuse, small discrete irregular red-brown macules of ogenesis. petechiae, the so-called ‘cayenne-pepper’ spots. Topical treat- ment is usually of limited value although a trial of potent top- ical steroids may be worthwhile. PUVA has also been reported to be successful in one series. PA-11 Pseudoxanthoma elasticum (PXE) presenting in childhood with unusual cutaneous lesions and dilated cardiomyopathy PA-10 L. Newell, C. Henderson and G. Theaker Congenital anetoderma in a preterm infant Southampton University Hospitals NHS Trust, Southampton, U.K. E.M. Wain, J.E. Mellerio, A. Kaiser* and D.J. Atherton A 12-year-old girl presented with a short history of breathless- St John’s Institute of Dermatology and *Department of Paediatrics, St. Thomas’ ness, chest pain and lethargy. There was no preceding history Hospital, London, U.K. of viral or systemic illness and there was no relevant family At birth our patient was noted to have patches of atrophic skin history. Systems examination revealed raised JVP, ascites, jaun- resembling scars on her abdomen, extending into the axillae dice and hepatosplenomegaly. Echocardiography demonstrated bilaterally. She was born prematurely, at 24 weeks’ gestation severe biventricular dysfunction, and dilated cardiomyopathy weighing 640 g. She was initially one of a twin pregnancy was diagnosed. Cardiomyopathy and autoimmune screens but her twin underwent feticide at 15 weeks’ gestation due to were negative. She was noted by the admitting team to have a chromosomal abnormality; the pregnancy was otherwise ‘punched out’ lesions on her face and a dermatology opinion uncomplicated with no evidence of maternal infection. During was sought. On examination she had discoid, crusted lesions her stormy postnatal period she was treated for respiratory on her forehead and cheeks with pale, atrophic scars. There distress, multiple apnoeic episodes, repeated episodes of sep- were also hyperkeratotic, papular lesions on her elbows and sis, gastroesophageal reflux, poor feeding and a patent ductus knees which looked excoriated. A skin biopsy taken from her arteriosus. As her other problems resolved, it was noted that knee showed perforating collagenosis, prompting a second her cutaneous lesions were extending as she grew but were biopsy, taken from nonlesional axillary skin. This demonstra- not increasing in percentage body surface area affected; a ted degenerate, fragmented elastic fibres in the middle and dermatology opinion was therefore sought at 4 months of deep dermis, characteristic of pseudoxanthoma elasticum age. On examination she had well-demarcated, translucent, (PXE). Due to progressive deterioration in her cardiac function annular, coalescing atrophic patches with wrinkling of the skin she was referred to a tertiary paediatric centre for urgent car- on the central and lateral aspects of her lower abdomen, diac transplantation. Myocardial histology revealed clumped, extending into the axillae bilaterally; elsewhere her skin was fragmented elastic tissue in aorta and pulmonary arteries, con- normal. There was no epidermal involvement and no history firming the diagnosis of PXE. PXE is an inherited disease of or evidence of inflammation. A skin biopsy demonstrated atro- connective tissue affecting the skin, retina and cardiovascular phy of the dermis with reduction in elastic fibres confirming system, characterized by elastic fibre mineralization and frag- the clinical diagnosis of anetoderma. Anetoderma is a rare mentation. Classically, skin changes develop in the second or cutaneous disorder characterized by circumscribed areas of third decade of life, with yellowish papules arising around the decreased dermal elastic fibres. It may occur as a primary idio- neck, below the clavicles and in the axillae, giving rise to a pathic phenomenon or secondary to a number of inflamma- ‘chicken skin’ appearance. Unusually, there may be spontan- tory conditions or penicillamine ingestion. Anetoderma, eous perforating lesions, which present as hyperkeratotic occurring within the first year of life, in premature infants papules, as in our patient. Cardiovascular complications (born at 24–29 weeks’ gestation) has been described as a sep- include claudication, hypertension, angina and myocardial arate entity possibly related to the use of cutaneous monitor- infarction. Mitral valve prolapse and restrictive cardiomyopa- ing leads or adhesives (Prizant TL, Lucky AW, Frieden IJ et al. thy are other rare associations. To our knowledge there is only Spontaneous atrophic patches in extremely premature infants. one reported case of dilated cardiomyopathy in association Anetoderma of prematurity. Arch Dermatol 1996; 132: 671–4). with PXE (Lebwohl M, Halperin J, Phelps RG. Occult pseudo- Congenital anetoderma has been reported once previously in elasticum in patients with premature cardiovascular preterm twins born at 25 weeks’ gestation (Zellman GL, Levy disease. N Engl J Med 1993; 329: 1237–9). Detection is import- ML. Congenital anetoderma in twins. J Am Acad Dermatol 1997; ant because many of the complications of PXE can be avoided, 36: 483–5). However, the lesions in these cases were limited and genetic counselling can be offered to family members of to a single 4 x 3 cm area of anetoderma in the first and two affected patients. Variable expression of the disease can present 2 x 2 cm areas in the second twin. Our patient presented with diagnostic difficulty; however, dermatological assessment may anetoderma covering approximately 10% of the body surface inform the diagnosis before serious systemic or ocular compli- area. We present the clinical and histological features of this cations develop in adulthood.

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PA-12 PA-13 Topical albendazole in the management of Chronic granulomatous disease presenting at 6 cutaneous larva migrans years of age S.K. Siddha and C. Jury P. Hampton, S. Brown and A. Taylor Royal Hospital for Sick Children, Glasgow, U.K. Royal Victoria Infirmary, Newcastle Upon Tyne, U.K. An 8-year-old girl was referred with an 8-week history of an A 6-year-old boy presented to dermatology with a 2-month itchy rash on the buttocks and left thigh. The eruption began history of swelling of the scrotum and penis. A previous diag- after a holiday in Kenya during which time the child had been nosis of cellulitis had been made by the general practitioner, swimming in salt and fresh water, spent time on the beach but there had been no improvement despite 7 days of oral and had been on safari. There was no history of a preceding flucloxacillin 250 mg q.i.d. Past medical history included one sting or insect bite. The first presentation was to A&E where a perianal abscess aged 2 years. He was otherwise healthy with diagnosis of urticaria was made. Treatment with topical beta- normal growth and development. On examination there was methasone cream (Fucibet; LEO Pharma, Princes Risborough, erythema and swelling of the scrotum and penis and erythema U.K.) and oral antihistamines was of no benefit. On presenta- and fissuring in the perianal area. Palpation revealed indura- tion to dermatology she had multiple excoriations across the tion of the scrotum and bilateral groin lymphadenopathy. A buttocks, extending down the left thigh. Subtle erythematous skin biopsy from the scrotum showed extensive dermal serpiginous tracks were noted at the lower pole of activity and inflammatory infiltrates with lymphocytes, histiocytes, well- a clinical diagnosis of cutaneous larva migrans (CLM) was formed noncaseating granulomata, and numerous multinucle- made. After discussions with colleagues in infectious diseases, ate giant cells. Routine blood tests were normal. A nitro-blue treatment with 10% albendazole ointment in yellow soft par- tetrazolium test for neutrophil function was abnormal and a affin was commenced. The treatment was applied 3 times neutrophil flow cytometric dihydrorhodamine oxidative burst daily for 10 days. Within 7 days the intense itch had resolved test showed markedly reduced neutrophil fluorescence consist- and by 6 weeks the acute rash had cleared leaving postinflam- ent with a severely reduced oxidative burst. Maternal neutro- matory staining only. There has been no recurrence. CLM is a phil function tests showed a dual population of neutrophils cutaneous lesion produced by percutaneous penetration and and therefore a diagnosis of presumed X-linked chronic gra- migration of larvae of various nematodes, mainly Ancylostoma nulomatous disease (CGD) was made. Further investigations braziliense, A. caninum, A. duodenale and Necator americanus. CLM char- showed absence of the GP91 subunit of the PHOX protein, acteristically presents with erythema and serpiginous tracks part of the nicotinamide adenine dinucleotide phosphate caused by parasite migration. Feet, hands and buttocks are (NADPH) oxidase system, confirming the diagnosis. Treatment commonly involved and people at risk include plumbers, pest was commenced with prednisolone 1 mg kg–1 daily, raniti- controllers, farm workers, sea bathers and children playing in dine 25 mg b.d., cotrimoxazole 240 mg b.d. and itraconazole sand. Itch is the main symptom and subsequent vigorous 5mgkg–1 o.d. Subsequently, the genital oedema has partially excoriation can obscure the clinical signs as in our case. In the reduced and he has developed no infective complications. U.K. treatment of CLM is commonly with oral ivermectin or CGD is caused by a defect in the neutrophil NADPH (reduced albendazole (Caumes E, Carrieri J, Datry A et al. A randomized form) oxidase enzyme. This results in an inability of neutro- trial of ivermectin versus albendazole for the treatment of phil phagocytes to produce bactericidal superoxide anions ) cutaneous larva migrans [Letter]. Am J Trop Med Hyg 1993; 49: (O2 ) and a subsequent susceptibility to recurrent bacterial 641– 4). However, these drugs are only available on a named and fungal infections. This leads to the characteristic accumu- patient basis and are relatively contraindicated in young chil- lation of granulomata and sepsis including skin infections, dren. Topical application of 10–15% thiabendazole ointment pneumonia, lymphadenitis, enteritis and hepatic, splenic or to the affected area is effective but thiabendazole is no longer perianal abscesses. The NADPH oxidase enzyme exists as a available. This prompted us to try topical albendazole, which complex of five component proteins. Mutations of any com- has been reported to be effective in one case report (Caumes ponent may lead to CGD but mutations in the GP91 subunit E. Efficacy of albendazole ointment on cutaneous larva migrans are most common. Therapy includes prophylaxis against and in two young children. Clin Infect Dis 2004; 38: 1647–8). Our treatment of infections. IFN-c reduces the frequency of infec- patient reported that the topical preparation has a grainy tex- tions but the only curative therapy is haemopoietic stem cell ture when applied but there were no adverse effects. We sug- transplantation. The presentation in this boy is unusual since gest 10% albendazole in yellow soft paraffin should be most cases present before the age of 5 with bacterial and fun- considered a safe and effective first-line treatment for children gal infections. The prognosis is worst in the X-linked group with CLM. with a high mortality in the second and third decades. The apparently mild phenotype in this case may confer a more favourable prognosis.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 British Society for Paediatric Dermatology 125

PA-14 PA-15 Multiple nutritional deficiencies mimicking erythro- A haematological presentation of emerging systemic poietic protoporphyria lupus erythematosus. Is this more common than we S.K. Siddha, P. McHenry, M. O’Reagan, P. Galloway think? and M. Badminton* J. Angus and R. Murphy Royal Hospital for Sick Children, Glasgow, U.K. and *University Hospital of Queen’s Medical Centre, Nottingham, U.K. Wales, Cardiff, U.K. We present a case of a 6-year-old boy, referred to the haema- A 10-year-old boy was referred to us while an inpatient for tologists, with persistent neutropenia (< 0Æ1x109 L–1) and evaluation of a chronic skin eruption predominantly affecting mouth ulcers. He subsequently developed a paronychia of his his face. He had been admitted to the medical ward following right index finger which required intravenous antibiotics and development of progressive difficulty walking and increased surgical drainage. To investigate the neutropenia a bone mar- irritability. Six months previously he had been admitted with row aspirate was performed which was normal and a diagno- a severe respiratory tract infection. His condition had generally sis of idiopathic neutropenia was made. Treatment with daily deteriorated since that time with exacerbation of a skin erup- subcutaneous granulocyte colony-stimulating factor (G-CSF) tion. He had been diagnosed with atopic eczema at the age of was commenced. At injection sites he developed intermittent 2 months and managed with intermittent topical steroids. At bruising and a more widespread urticarial rash which was the age of 6 he attended the immunologists with a history of attributed to G-CSF treatment. There was no history of arthral- reacting to milk and egg. Investigations with RAST and skin gia, arthritis or significant past medical illnesses. A family his- prick tests were positive to milk, egg white and nut and tory of idiopathic affecting a advice was given regarding avoidance of these. On examin- maternal aunt was noted. There was good response to treat- ation he had peeling and crusting of his lips and erythematous ment but neutrophil counts decreased if the G-CSF was omit- patches in a photosensitive distribution affecting the cheeks, ted which was also associated with a transient tip of his nose and neck. Neurologically, he was withdrawn thrombocytopenia. He remained well until December 2006, and irritable and his plantar response and deep tendon reflexes when he was referred to the dermatologists with a new rash were absent. Investigations showed increased erythrocyte and affecting his face and ears. There had been three episodes of plasma free protoporphyrin (PP) levels consistent with ery- this eruption over the last 12 months. On examination there thropoietic porphyria (EPP) although the levels were some- were well-demarcated small necrotic lesions on both malar what lower than usual for this condition. Mutation analysis of surfaces and pinnae. The differential diagnosis included cryo- the ferrochelatase (FECH) gene did not show any abnormality. globulinaemia, thrombophilic tendency, systemic lupus

Serum zinc, vitamins C, B6,B12, red cell folate and free carni- erythematosus (SLE) or G-CSF-induced vasculitis. There was tine and ferritin levels were all low. Following these results, it no evidence of other skin lesions suggestive of SLE or emboli transpired that he was also avoiding all wheat and gluten affecting the digits. Investigations revealed a low C3 of 0Æ880 products because of his autism. His irritability, neurological gL–1 (0Æ9–1Æ8) and positive lupus anticoagulant antibody. and facial rash resolved and erythrocyte free PP levels normal- Antinuclear antibodies, double-stranded DNA and cryoglobu- ized after vitamins and trace elements were administered. lins were negative. A biopsy showed small dermal vessel Children on restricted diets for food allergies or other medical occlusion with no evidence of vasculitis consistent with a reasons are at increased risk of nutritional deficiencies (Fox thrombophilic tendency. A clinical presentation of recurrent AT, Du Toit G, Lang A, Lack G. Food allergy as risk factor for mouth ulceration in the context of persistent neutropenia, nutritional rickets. Pediatr Allergy Immunol 2004; 15: 566–9). The thrombocytopenia, low complement and positive lupus anti- skin eruption was unusual, particularly with the involvement coagulant antibodies is highly suggestive of a diagnosis of SLE. of the tip of his nose. In EPP patients, deficiency of FECH, the This case highlights the unusual and diverse presentations of final enzyme in the haem biosynthetic pathway, results in the SLE in the paediatric population (Iqbal S, Sher MR, Good RA, accumulation of free protoporphyrin, giving rise to symptoms Cawkwell GD. Diversity of presenting manifestations of sys- of acute photosensitivity. In iron-deficient patients, FECH temic lupus erythematosus in children. J Pediatr 1999; 135: activity is normal and catalyzes the insertion of the divalent 500–5) and emphasizes the importance of early diagnosis and cation zinc into protoporphyrin, resulting in increased eryth- investigation. SLE should always be considered as a differential rocyte zinc protoporphyrin, which is readily distinguishable diagnosis, if haematological abnormalities of leucopenia, from free protoporphyrin. We propose that in our patient the lymphopenia or thrombocytopenia are identified. Neutropenia marked deficiency of both zinc and iron, in the presence of as an isolated haematological finding and presenting feature of normal ferrochelatase activity, resulted in accumulation of free SLE is unusual, but is significant as it predisposes to the devel- PP levels usually associated with EPP, and which normalized opment of severe and potentially life-threatening infections. following nutritional supplementation. There should be increasing awareness of unusual presentations of SLE amongst dermatologists, rheumatologists and haematol- ogists, and appropriate investigation considered before both diagnosing and treating isolated neutropenia as idiopathic.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 126 British Society for Paediatric Dermatology

PA-16 PA-17 Childhood bullous pemphigoid: a rare disease with Infantile pyoderma gangrenosum management challenges M.A. McAleer, F.C. Powell, D. Devaney* V. Majmudar, D. Herath, E.A. O’Toole* and and B.F. O’Donnell* A.P. Harrison The Regional Centre of Dermatology, Mater Misericordiae University Hospital, Mid Essex Hospitals NHS Trust, Essex, U.K. and *Barts and the London Dublin, Ireland and *The Children’s University Hospital, Temple Street, Dub- NHS Trust, London, U.K. lin, Ireland A 10-week-old white girl presented with a 10-day history of Pyoderma gangrenosum (PG) is unusual in children and vesicles and blisters on her palms and soles. Of note, she had infants. There are only 12 cases of PG in infants (less than 1 her first routine vaccinations 1 week prior to onset of blisters. year of age) reported. Eight of these cases have been con- Her brother and grandmother were recently treated for impe- trolled with systemic steroids and one with topical steroids tigo. She was commenced on antibiotics, with a provisional alone. Eight of the reported patients with infantile PG have diagnosis of bullous impetigo. Examination revealed an alert had an underlying medical condition. We report a case of an and apyrexial infant with multiple, tense serous and serosan- otherwise well, 8-month-old, boy with PG. This infant’s PG guinous blisters of differing sizes on her hands and feet. was aggressive and uncontrolled despite high-dose oral ster- Erythematous annular plaques on her face, abdomen and inner oids. Additional treatment with ciclosporin was necessary to thighs were present. There was no scarring or milia. Nikol- achieve healing. An 8-month-old, identical twin, boy presen- sky’s sign was absent. A skin biopsy showed an intact subepi- ted to the surgical department with an ulcer on his left thigh. dermal blister containing fibrin, neutrophils and eosinophils The ulcer was 5Æ5x3Æ5 cm with a necrotic centre and a deep- with minimal lymphocytic perivascular infiltrate. Direct immu- ly erythematous border. It began as a pustule at a site of nofluorescence (IMF) showed strong staining of the basement minor trauma and rapidly progressed over a 2-week period, membrane zone (BMZ) for IgG and C3 but no staining for despite treatment with intravenous antibiotics. The ulcer was IgA and IgM, supporting a diagnosis of bullous pemphigoid. excised. At 1 day after operation two small lesions on the con- Indirect IMF also showed positive staining at the BMZ. Antibi- tralateral thigh were observed and a dermatology opinion was otics were discontinued and after initial improvement with sought. The infant was pyrexic and distressed. The excision betamethasone ointment (Fucibet; LEO Pharma, Princes Ris- site was erythematous and violaceous. There were vesicopus- borough, U.K.) she developed new blisters. Prednisolone at tules on the right thigh and on the ankles and antecubital fos- 1mgkg–1 was commenced with good initial response. On sae at sites of venepuncture. The clinical impression was of review, once again, she had deteriorated, with more extensive PG. The histopathology was supportive of this diagnosis. There involvement of her scalp and trunk. Tense bullae and annular were no pathogenic organisms cultured. Normal serological plaques were noted periorbitally and in genital areas. Mucous investigations included: full blood count, renal, liver and bone membranes were not affected. The prednisolone was increased profile, oxidative burst test, lymphocyte subsets, immunoglo- to 2 mg kg–1. Clobetasol propionate (Dermovate; Glaxo- bins, autoantibodies, complement and complement fixation SmithKine, Uxbridge, U.K.) ointment and erythromycin were and a HIV test. Other normal investigations included stool for added with resolution of her blisters within 2 weeks. Her occult blood, blood cultures, chest X-ray and skin swabs. The prednisolone is being weaned slowly by 0Æ5 mg per week but C-reactive protein was raised at 108 mg L–1. Treatment was she continues on the erythromycin and Dermovate. She commenced with oral prednisolone at 2 mg kg–1 daily with remains under close follow-up. Bullous pemphigoid (BP) of an initial response. After 13 days of steroid therapy the childhood is rare. It was first described as a clinical and histo- infant’s condition deteriorated and the ulceration became pathological entity by Lever in 1953. There is no racial or sex- inflamed and started to extend. Spontaneous vesicopustules ual predilection. Marked involvement of the palms and soles is developed at widespread sites. The infant was in acute distress. the hallmark of clinical presentation in this age group. The The prednisolone was increased to 3 mg kg–1 daily. However, cause is unknown although cases have been described related this did not control the disease. Systemic ciclosporin was com- to various vaccines. The age of onset varies between 2 months menced at a dose of 5 mg kg–1 daily. There was a rapid clin- and 14 years making ours one of the youngest cases. Treat- ical response within 3 days. The lesions healed following 6 ment options include oral prednisolone at 1–2 mg kg–1.In weeks of ciclosporin therapy. After 12 weeks of ciclosporin severe cases, adjunctive therapy with dapsone, sulfapyridine the dose was slowly reduced, and it continues to be weaned. or, more rarely, erythromycin has been reported. Childhood The follow-up in previously reported infantile PG cases range BP often follows a benign course. Disease duration of less than from 25 days to 5 years. In some cases the PG predated the 1 year is frequently cited. We report a case of childhood BP onset of other medical conditions. We present the first repor- with characteristic clinical, histopathological and immunologi- ted case of infantile PG successfully managed with ciclosporin. cal features. To our knowledge, this is one of the youngest patients reported, who proved to be a management challenge. We propose that the BP may have been triggered by a vaccin- ation given a few days prior to the onset of blisters.

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PA-18 PA-19 Ecthyma gangrenosum: an important sign of Unilateral cutis marmorata telangiectatica congenita potentially fatal Pseudomonas sepsis and a rare with skin necrosis case occurring in a previously well child C.V. DeGiovanni, S.B. Syed,* J.R. Felton, S. Goolamali, A. Fogo, H. Shaikh, N. Brathwaite, P.N. Amess and C.R. Darley M. Ford-Adams and S. Macfarlane Department of Dermatology, Brighton and Sussex University Hospitals NHS King’s College Hospital, London, U.K. Trust, Brighton, East Sussex, U.K.; *Department of Dermatology, Great A previously healthy, 28-month-old Afro-Caribbean boy was Ormond Street Hospital, London, U.K.; and Department of Paediatrics, Brigh- admitted with a 4-day history of fever, diarrhoea and increas- ton and Sussex University Hospitals NHS Trust, Brighton, East Sussex, U.K. ing lethargy with multiple eroded lesions over the trunk and A female infant was delivered at 38 weeks’ gestation by Cae- limbs. The child was unwell and dehydrated and the cutane- sarean section. At birth striking vascular lesions were noted on ous features were striking, ranging from a small vesicular pa- the left lower limb and left abdomen. These comprised per- pule to multiple large (3–4 cm), well-demarcated annular/ sistent cutis marmorata, telangiectasia and phlebectasia. An ovoid plaques with dusky, violaceous erythema surrounding a ulcerated area of skin was noted within an area of bony prom- dry central necrotic area. There was a distinctive bullous edge inence just above the medial malleolus. Examination was oth- with significant induration of the underlying tissue. Investiga- erwise unremarkable. Purpura fulminans was initially tions revealed mild anaemia (Hb 10Æ7) with a low normal suspected and she was treated with fresh frozen plasma and white cell count (WCC 4Æ88, neutrophils 2Æ06) and thrombo- vitamin K. A thrombophilia screen was negative and this diag- cytopenia (Plt 92). He had a markedly raised CRP (436) and nosis was excluded. A dermatology opinion was sought and a ESR (94) with impaired renal function (urea 22, creatinine diagnosis of cutis marmorata telangiectatica congenita (CMTC) 151). He was treated empirically for sepsis and commenced was made. The ulcerated area was dressed with Mepitel on broad-spectrum antibiotics (benzyl penicillin, flucloxacillin, (Mo¨lnlycke, Dunstable, U.K.), mupirocin ointment (Bactro- gentamicin and ceftazidime). Twenty-four hours later the ban; GlaxoSmithKline, Uxbridge, U.K.) and Sorbsan (Uno- lesions had become more inflammatory and necrotic, consist- medical, Redditch, U.K.). Skin swabs and blood cultures were ent with bullous pyoderma gangrenosum and oral predniso- negative. Further investigations included cranial ultrasonogra- lone was added. A skin biopsy from one of the early lesions phy that revealed no congenital anomaly. Abdominal ultra- on the trunk supported this diagnosis with marked vesicula- sound and echocardiography were normal. She was discharged tion of the epidermis and extensive underlying dermal necro- home with community paediatric nurse support. At 3-months sis. However, an urgent skin microscopy showed Gram follow-up she was thriving with no developmental delay. negative rods, and both skin and blood cultures were positive There was no limb length discrepancy and the ulcerated area for Pseudomonas aeruginosa. An unusual feature of the lesions was was healing. CMTC is an uncommon condition that most lack of suppuration and slough and they progressed to form a commonly affects the lower limb of neonates. It can be associ- well-demarcated eschar which is typical of ecthyma gangreno- ated with skin atrophy and necrosis as in our case. CMTC has sum secondary to Pseudomonas sepsis. He responded well to i.v. been associated with hypertrophy or hypoplasia of the affected antibiotics and the lesions improved with conservative man- limbs, vascular anomalies, glaucoma and macrocephaly; how- agement and complete healing at 2 months. He has significant ever, in the majority of cases no associated congenital anomaly scarring but remains well. Investigations have not revealed any is noted (Amitai DB, Fichman S, Merlob P et al. Cutis marmo- underling cause (HIV-negative). Ecthyma gangrenosum is a rata telangiectatica congenita: clinical findings in 85 patients. rare cutaneous manifestation of P. aeruginosa septicaemia. The Pediatr Dermatol 2000; 17: 100–4). The cutaneous lesions tend lesions are characteristic and the condition has a high mortal- to improve with age. The aetiology of the condition is ity unless treated early. It usually occurs in neutropenic or unknown although some authors suggest a teratogenic cause immunocompromised individuals but occasional cases have or an autosomal dominantly inherited disease with incomplete been reported in healthy children. It seems extremely rare in penetrance (Amitai et al.). Other authors have suggested that the U.K., with reports originating from Southeast Asia. Young the Happle lethal gene hypothesis (that the lethal gene sur- children are most commonly affected with fever and diarrhoea vives by means of mosaicism) may explain the patchy distri- as recognized associations. (Huang YC, Lin TY, Wang CH et al. bution of the lesions. The striking segmental arrangement in Community-acquired Pseudomonas aeruginosa sepsis in previously our patient would lend support to this theory. healthy infants and children: analysis of forty-three episodes. Paediatr Infect Dis J 2002; 21: 1049–52; Duman M, Oxdemir D, Yis U et al. Multiple erythematous nodules and ecthyma gang- renosum as a manifestation of Pseudomonas aeruginosa sepsis in a previously healthy infant. Paediatr Dermatol 2006; 23: 243–6).

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 128 British Society for Paediatric Dermatology

Posters

PA-20 PA-21 An audit of the activities of the paediatric Visual outcomes of treated periocular capillary dermatology nurse specialist (PDNS) in the Leeds haemangiomas in childhood: a 10-year review General Infirmary S. Gawley, A. Bingham and G. McGinnity N. Usmani and S. Clark Royal Victoria Hospital, Belfast, U.K. Leeds General Infirmary, Leeds, U.K. Capillary haemangiomas are the commonest orbital and eyelid The effectiveness of many therapies in dermatology is directly tumours of childhood. As well as cosmetic disfiguration they related to patient compliance. In the paediatric population this can result in amblyopia. Rates of 43–60% have been reported remains a key factor in treatment success with the paediatric (Stigmar G, Crawford JS, Ward CM, Thomson HG. Ophthal- dermatology nurse specialist (PDNS) serving an instrumental mic sequelae of infantile hemangiomas of the eyelids and role to achieve this. In the current financial climate where orbit. Am J Ophthalmol 1978; 85: 806–13; Haik BG, Jakobiec resources in the National Health Service are becoming increas- FA, Ellsworth RM, Jones IS. Capillary hemangioma of the lids ingly limited it is essential that the role of the PDNS remains and orbit: an analysis of the clinical features and therapeutic appreciated and supported. We present the results of an audit results in 101 cases. Ophthalmology 1979; 86: 760–92). Treat- conducted in the dermatology department of the Leeds General ment is usually instituted where there is actual or potential Infirmary (LGI) highlighting the significant and beneficial role visual impairment. This study considers visual and cosmetic of our PDNS. In the period between January 2005 and January outcomes in such cases. Patients who received inpatient treat- 2006 the PDNS worked 21Æ5 h over a 4-day week. Details of all ment for periocular capillary haemangioma between August activities performed by the PDNS in this time period at the LGI 1996 and August 2006 inclusive were identified by the hospi- were retrieved from a comprehensive computer database and tal data collection system. A retrospective chart review was pertained to the following: visits made outside of hospital then carried out. Seventeen patients were identified. The age (home visits, school visits, GP), ward visits, patients seen in the range at first clinic visit was 4 weeks to 5 months. There were outpatient department (OPD), patient liaison by mail or tele- 13 female and four male patients. Twelve had lid haemangio- phone and teaching activities. Seventy-nine visits were per- mas, four had lid with orbital involvement and one had an formed outside the hospital 78% of which were home visits for extensive facial and periocular haemangioma. The main indi- those children suffering from eczema. Such visits helped to cations for treatment were increasing size of lesion, threatened ensure treatment compliance within the confines of the home or actual visual occlusion. Eight patients received intralesional environment as well as providing an opportunity for further steroids, three had systemic steroids and six had more than education. Eleven per cent of visits were performed for children one type of treatment. The patient with the extensive facial with allergies where the PDNS reiterated the importance of haemangioma required numerous treatments including inter- avoidance of potential allergens as well as performing one to one feron alfa-2a. The only complication of intralesional steroid teaching of Epipen (ALK-Abello Ltd, Hungerford, U.K.) training. treatment was slight bruising in one patient. Of six patients Nine educational service visits were made of which 66% were in treated with oral steroids four had side-effects; interferon alfa- relation to allergy and 34% to eczema. Thirty ward visits were 2a side-effects included fever and aching. In all cases the le- undertaken to inpatients of which just under two-thirds (60%) sion reduced in size with clear visual axis. Five patients had were for children with eczema. A 30% reduction in inpatient anisometropic astigmatism (one dioptre or more) pretreat- admissions was recorded in this time period. A survey of outpa- ment, which reduced in four cases but increased in one. Two tient activity revealed that the PDNS saw 376 patients over the other patients later developed anisometropic astigmatism. Four 12 months equivalent to 28Æ8% of all follow-ups seen in the pae- patients developed strabismus, one patient had persistent stra- diatric dermatology clinic. As a result of this consultants and bismus and in one it resolved. There were 16 patients at risk their juniors were able to see more than twice as many new of or diagnosed as having amblyopia and after treatment this patients (256 patients in 2001 compared with 585 patients in reduced to six patients, (three unknown); only two patients 2005). The PDNS also made a significant contribution to both had vision worse than 6/9 and the deepest amblyope was 6/ liaison work and teaching. Ten dermatology-based teachings 18. Treatment of periocular capillary haemangiomas should be were delivered over the 12 months, five in relation to eczema considered where there is a risk of visual loss, the other and five related to allergies in children. One hundred and indication is when the lesion is particularly large. Treatment twenty-two professionals attended these teachings, 80% of results in regression of the lesion and reduces the long-term whom attended the lecture on eczema. In conclusion our audit significant ocular sequelae. has shown that the PDNS is an integral part of the dermatology team offering a seamless package of care to patients in the com- munity and at home leading to a decrease in hospital admissions as well as providing a significant positive impact on waiting lists.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 British Society for Paediatric Dermatology 129

PA-22 PA-23 Congenital cutaneous candidiasis an under- Dietician review in paediatric dermatology: who recognized condition? benefits? S. Kamath, J. Hughes and N. D’souza P. Balasubramaniam, K. Shalders and A. Abdullah Princess of Wales Hospital, Bridgend, U.K. Birmingham Skin Centre, Birmingham, U.K. We describe a 3060-g female infant delivered by emergency It is not uncommon for parents to blame a food allergy as a section for fetal distress to a 35-year-old white primipara at contributing factor towards their child’s skin disease. Johnson 40 weeks of gestation. The mother had a urinary tract infec- et al. found that 75% of parents of children referred to second- tion at 31 weeks and developed vaginal candidiasis after a ary care with a diagnosis of atopic eczema had tried dietary course of antibiotics. The pregnancy was otherwise uncompli- manipulation to improve their child’s eczema (Johnston GA, cated. At birth there was thick meconium present and the Bilbao RM, Graham-Brown RA. The use of dietary manipula- infant had respiratory failure treated with ventilation for 44 h tion by parents of children with atopic dermatitis. Br J Dermatol after birth. Immediately after birth the infant was noted to 2004; 150: 1186–9). Only half of these had consulted a doc- have a widespread macular, erythematous rash, which tor or dietician before making changes and 39% of parents felt involved the whole body. Fine desquamation was noted in the that the changes had benefited their child’s skin disease. We periorbital area on day 2. A clinical diagnosis of toxic pustulo- performed a retrospective review of children who were sis secondary to prolonged contact with meconium and referred to a dietician from a paediatric dermatology clinic extreme humidity was considered and mild topical steroid– over a 2-year period (2004–2005), to study the impact of a antiyeast cream was prescribed. Multiple skin swabs and a full dietician consultation on skin disease. Fifty children were infection screen were carried out. The rash became more referred, 28 males and 22 females. Twenty-six patients were florid and confluent on day 3 and the infants physical condi- of Indian subcontinent origin (12 Indian, 12 Pakistani, two tion deteriorated with an episode of pyrexia of 38Æ1 C. The Bangladeshi), nine white, 11 Afro-Caribbean and four other infant was started on intravenous flucloxacillin and gentami- groups. Atopic eczema was the diagnosis in 46 children cin. Blood counts, LFT, U&E and IgGs were normal. The septic (92%), while two had orofacial granulomatosis, one had urti- screen (blood, urine and CSF) was negative after 5 days. By caria/angio-oedema and one child did not have any documen- day 5 a diagnosis of congenital cutaneous candidiasis was con- ted diagnosis. Advice regarding possible food allergy was the sidered and this was supported by growth of Candida species main reason for referral in 39 patients (78%). The other rea- (including C. albicans) from swabs of eye, neck, groin and sons included elimination diet, intolerance to milk and poor mouth. Clotrimazole cream topically and nystatin oral suspen- weight gain. No record was found in five children. Thirty- sion were prescribed. Systemic anticandidal treatment was not nine children (78%) attended consultation with a dietician. Of given as her physical condition improved and the temperature those, 22 patients (56%) only had a single consultation. Nine returned to normal by day 5. The rash gradually improved patients (23%) reported improvement following the dietary and resolved with widespread desquamation. At the time of advice. There was no documentation of any benefit in 25 chil- review in clinic at week 6 the skin had improved significantly dren (64%). These children continue to have moderate to with only a few areas of desquamation and erythema. Beau’s severe active atopic eczema. Five patients did not attend fol- lines were present. The infant was otherwise thriving. Topical low-up. Dietician input benefited a quarter of our patients treatment was discontinued. The most common presentation where food allergy was suspected as a contributing factor of candidal infection in the newborn is oral candidiasis and towards their skin disease. To avoid costly excess referrals a the mode of infection is via passage through the birth canal. large-scale prospective case–control study would be helpful to Given the common occurrence of vaginal candidiasis in preg- establish which subgroup of patients benefits from the input nancy it is remarkable that this condition is so uncommon. of a dietician. Contrary to the current literature it seems unlikely that this condition (Priori G, Marenzi C, Escobar R et al. Three cases of congenital cutaneous candidiasis. Pediatr Med Chir 2004; 26: 53–6, in Italian; Diana A, Epiney M, Ecoffey M, Pfister RE. ‘White dots on the placenta and red dots on the baby’: con- PA-24 genital cutaneous candidiasis – rare disease of the neonate. Acta Vitamin D deficiency rickets in an Asian child with Paediatr 2004; 93: 996–9) is under-recognized. lamellar ichthyosis S. Dhoat, B. Laguda, J. Harper, C. Brain and M. Glover Great Ormond Street Hospital for Children, London, U.K. A 5-year-old Asian boy with severe ichthyosis, developmental delay, poor growth, b-thalassaemia trait and pulmonary steno- sis, fractured his right femur in a road traffic accident in India in March 2004. A year later, he suffered a second fracture of the femur after slipping on rough ground. Plasma 25-OH

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 130 British Society for Paediatric Dermatology vitamin D level was found to be 5 nmol L–1 (normal range was also evidence of plantar keratoderma with punctate 15–100). Serum parathyroid hormone was 29Æ3 pmol L–1 keratoses on the palms. It was felt that the diagnosis was most (normal range 0Æ7–5Æ6 pmol L–1) and ionized calcium was in keeping with acral poikiloderma of Weary. The poikiloder- 2Æ18 mmol L–1 (normal range 2Æ22–2Æ51). He was treated matous diseases encompass a wide range of conditions with with alfacalcidol 0Æ75 mg daily, followed by calcitriol (Rocal- many differing systemic features. The main differential of acral trol; Roche, Welwyn Garden City, U.K.) 0Æ25 mg daily and poikiloderma of Weary is that of Kindler’s syndrome. most recently colecalciferol 3000 u daily. Biochemical normal- Although there are clearly distinctions between the two condi- ization and marked improvement in mobility was noted tions the current school of thought is that they may actually within 3 months. Generalized disorders of keratinization may represent a single diverse group with the acrokeratotic vesicu- predispose children to vitamin D deficiency and rickets (Tha- lobullous poikilodermas described by Weary representing the cher TD, Fischer PR, Pettifor JM, Darmstadt GL. Nutritional more benign form of the disorder. In the literature there have rickets in ichthyosis and response to calcipotriene. Pediatrics only been a handful of cases with features of acral poikiloder- 2004; 114: 119–23). In addition, suboptimal plasma 25-hyd- ma of Weary alone although other patients may have been roxy-colecalciferol levels have been reported in preschool misreported as Kindler’s syndrome. There are also a number Asian children in Britain (Lawson M, Thomas M. Vitamin D of reports, which describe patients who have considerable concentrations in Asian children aged 2 years living in Eng- overlap of the two conditions. In conclusion we have presen- land: population survey. BMJ 1999; 318: 28), possibly due to ted a pedigree of grandmother, mother and child with varying a combination of inadequate sunlight and dietary factors. We degrees of blistering, poikilodermatous change and keratotic suggest that children with ichthyosis should be monitored for papules which we feel is most in keeping with a diagnosis of vitamin D deficiency and supplementation initiated for those acral poikiloderma of Weary. with low levels.

PA-26 PA-25 Childhood pemphigus vulgaris and SLE: a case Acral poikiloderma of Weary in a child and a review report and review of the literature of the literature S. Rajpara, J. Hewitt and I. Auchterlonie* N. Usmani and S. Clark Aberdeen Royal Infirmary and Royal Aberdeen Children’s Hospital, Aberdeen, Leeds General Infirmary, Leeds, U.K. U.K. and *Royal Aberdeen Children’s Hospital, Aberdeen, U.K. Poikiloderma is a term used to describe the cutaneous triad of A 13-year-old boy was referred to us from paediatricians with atrophy associated with reticulate hyperpigmentation and a 6-week history of oral blisters and ulcers. The general exam- telangiectasia. A wide spectrum of poikilodermatous condi- ination revealed widespread oral ulceration involving soft pal- tions exist of which acral poikiloderma of Weary was first de- ate, buccal mucosa and tongue. This boy had an interesting scribed in ten members of a white family in 1971. Since this past medical history. During the first 3 years of his life he had time there has been a limited number of such cases described nephrotic syndrome, hypertension and recurrent infections in the literature although there are other reports of patients with positive ANA, anti-ds DNA, thrombocytopenia, low C3 who demonstrate some of the features of this condition. We and C4 and low IgG (IgG 2, 3 and 4). His mother was tested present a female kindred with acral poikiloderma of Weary. A negative for ANA and ENA. During the following 9 years his 6-month-old baby girl was referred to the paediatric dermatol- systemic lupus erythematosus (SLE) and nephrotic syndrome ogy clinic with a history of blistering affecting the digits and has been in remission despite persistently high ANA (negative limbs, which had begun 2 weeks after birth occurring inter- anti-ds DNA) and low C3 and C4. Investigations during his mittently every 2–3 days with no relation to sun exposure. present illness revealed normal flora on throat swab culture The patient’s mother had had a similar history of blistering and negative herpes simplex virus DNA PCR. Direct immuno- occurring a few weeks after birth, which had continued for fluorescence on oral biopsy was suggestive of pemphigus vul- 10 months. In addition she described a long history of warty garis and pemphigus antibodies were positive at 1 : 320. lesions on the hands, which had also been present in her own Initially he was treated with oral prednisolone 2 mg kg–1 with mother who had been adopted. On examination of the child almost complete healing of the ulcers. Addition of azathio- there were small healing blisters on the lower legs and dor- prine 75 mg daily (2 mg kg–1) allowed gradual reduction of sum of the feet with erythema extending up to the elbows. the prednisolone to 10 mg o.d. alternate days. However, sub- There were macular areas of hyperpigmentation adjacent to sequent flare of the oral disease and appearance of new lesions the nail folds with peeling of the toes but no milia, scarring over the scalp required an increase in the dose of the predn- or palmoplantar keratoderma. Oral blisters and erosions were isolone to 30 mg alternate days and azathioprine to 125 mg absent. On examination of the patient’s mother there was evi- daily. Betamethasone soluble tablets 500–1000 lg dissolved in dence of poikiloderma affecting the fingers, forearms and lat- 10–15 ml of water and held in the mouth for 4–5 min t.d.s. eral borders of the feet, with minimal telangiectasia. There were used as well. Over the next 4 months his pemphigus

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 British Society for Paediatric Dermatology 131 deteriorated, so azathioprine was stopped and mycophenolate All were adults except one 15-year-old girl (Kuchabal DS, Ku- mofetil was started at 250 mg b.d. and increased to 500 mg chabal SD, Pandit A, Nashi HK. Pemphigus vulgaris associated b.d. At present, his disease is stable and the oral lesions seem with systemic lupus erythematosus. Int J Dermatol 1998; 37: to be healing. Since the beginning of the treatment, his ANA 636–8). These reports show SLE or pemphigus can manifest and anti-ds DNA titres have been normal, while his C3 and first. A series of seven patients with coexistent SLE and pem- C4 levels have been low. He does not fulfil the criteria for the phigus vulgaris and follow-up of 3–18 years reports less diagnosis of SLE at present. There have been 13 case reports severe SLE in these patients compared with patients with SLE in the literature of coexistent pemphigus vulgaris and SLE only (Malik and Ahmed). It is difficult to explain development (Malik M, Ahmed AR. Dual diagnosis of pemphigus vulgaris of pemphigus vulgaris after the SLE has been in remission for and connective tissue. J Am Acad Dermatol 2006; 55: 699–704). a long time.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 119–131 British Photodermatology Group: Summaries of Papers

PD-1 PD-2 Reduced prevalence of polymorphic light eruption in Medium dose ultraviolet A1 phototherapy for the skin cancer patients treatment of sclerotic skin diseases S. Lembo, J. Fallon, P. O’Kelly and G.M. Murphy R. Rose, D. Turner, M. Goodfield and V. Goulden Beaumont Hospital, Dublin, Ireland Department of Dermatology, The General Infirmary at Leeds, Leeds, U.K. Failure of the immune system to downregulate and ignore Long wavelength (340–400 nm) ultraviolet A phototherapy ultraviolet (UV) induced antigens in human skin results in (UVA1) was first reported to be effective for the treatment of polymorphic light eruption (PLE). PLE is extremely prevalent localized scleroderma in 1995. Mechanisms for this effect in countries well north of the equator, reaching a prevalence include an increase in fibroblast collagenase activity and the of 14–20% in Northern Europe. Ultraviolet radiation is a com- induction of T-cell apoptosis. However this therapy is not plete carcinogen. It initiates and promotes cancers. The net widely available. High output metal halide sources required to effect of UV-induced immunosuppression is to permit the administer high dose UVA1 are restricted, in part by the cost expansion of UV-mutated clones of cells. Ultimately expansion of this equipment but also by installation. Fluorescent tube of such clones of cells (P53 patches) leads to skin cancer. cubicles, which are able to deliver low to moderate output With increased immune surveillance and resistance to immune UVA1, are significantly less expensive and could potentially be suppression following UV radiation exposure in PLE, one used by more phototherapy units. We present the results of might expect a protective effect of PLE against skin cancer and our experience with medium dose UVA1 in patients with conversely a reduced risk of PLE among skin cancer patients. sclerotic skin diseases, which show encouraging responses in We performed a prospective study on 221 patients with histo- these often challenging conditions. Nineteen patients with logically proven skin cancer, 100 patients known to have PLE sclerotic skin disease were treated with UVA1. The group con- and 224 control subjects matched for gender and age. Each sisted of nine patients with systemic sclerosis (six diffuse type participant had to answer a questionnaire aimed at establishing and three limited type), seven patients with localized sclero- personal and family history of skin cancer and photodermatoses. derma, and three patients with scleredema. Patients received Skin type and exposure to UVR were also documented. UVA1 three times per week to a maximum of 30 exposures. Logistic regression methods were used to determine skin can- Most patients received moderate dose UVA1 (30 J cm–2). Two cer and PLE outcomes. The prevalence of skin cancer (SC) was patients, however, were treated using higher doses (40 and slightly lower in PLE patients (4%) compared with that in 50 J cm–2). In patients with systemic sclerosis, the outcome controls (5%) (NS). Moreover the prevalence of PLE in people was measured using the modified Rodnan skin score and a self with skin cancer was 7%, compared to 21% for controls (odds reported patient global assessment by visual analogue scale ratio 0Æ29, P <0Æ001). In a multi-factorial model, reduced from 0 (no benefit) to 10 (complete response). For patients PLE for skin cancer patients was independently significant of with localized scleroderma and scleredema, the response was age, gender and sun exposure level (P <0Æ001). Our results graded using a global physician score as ‘poor response’ show strong evidence of reduced PLE in skin cancer patients 0–25% improvement, ‘fair response’ 25–50% improvement, as might be expected from studies by Palmer et al. (Palmer ‘moderate response’ 50–75% improvement and ‘good RA, Hawk JLM, Young AR, Walker SL. The effect of solar- response’ above 75% improvement. For localized scleroderma, simulated radiation on the elicitation phase of contact hyper- a patient global assessment score was also recorded. Most sensitivity does not differ between controls and patients with patients with systemic sclerosis responded well with a median polymorphic light eruption. J Invest Dermatol 2005; 124: 1308– improvement of 56% in the skin score (range 25–66). Patient 12). The 100 PLE patients were younger than the skin cancer global assessment showed a median score of 8 (range 2–9). In patients attending hospital. The size of this cohort of patients patients with localized scleroderma, four patients were may be insufficient to show a statistically significant difference assessed to have a good response, one a moderate response in skin cancer prevalence compared with controls. Having PLE and two poor responses. The median patient global assessment may therefore protect against skin cancer despite equivalent score was 8 (range 2–10). In the patients with scleredema, UV exposure. the outcome was graded as fair in two patients and moderate in one. Side-effects occurred in three patients. Two patients developed erythema and one patient polymorphic light erup- tion. Long term side-effects associated with this therapy are not yet established. Our results have shown significant benefit

2007 The Authors 132 Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 132–138 British Photodermatology Group 133 in conditions for which there are currently few other effective disease phenotype. These individuals have survived, albeit therapies. PUVA remains the treatment option available to with marked involvement, into adult life without any definit- most dermatologists and a study comparing this therapy to ive treatment or consistent photoprotection. UVA1 is required. Two patients developed mild treatment- induced erythema, though one had co-existent vitiligo, and one patient developed polymorphic light eruption. In all patients experiencing improvement, the benefits were main- tained for a minimum of 3 months. PD-4 A new combination treatment of vitiligo I. Sheikh Mayo Hospital, Lahore, Pakistan Vitiligo is an ever increasing problem in all age groups in our PD-3 country. Different regimens are used, including sun exposure, Congenital erythropoietic porphyria: a novel muta- UVR, oral and topical psoralens. This paper presents original tion work with a new idea where needling is combined with nar- S. Walker, M. Shah,* S. Whatley and row band ultraviolet (UV)B exposure. Our hypothesis is that M. Badminton surrounding epidermal cells (including melanocytes) can be London School of Hygiene and Tropical Medicine, London, U.K.; *Anandaban pushed or dragged from the edge of normal skin into a patch Hospital, Kathmandu, Nepal and Porphyria Service, UHW Cardiff and Vale of vitiligo; subsequently UVB-induced melanogenesis treats NHS Trust, Cardiff, U.K. the vitiligo. A 30 G needle with a short stem is gently pushed A 37-year-old Nepali woman presented to the leprosy service through the edge of normal skin into the patch of vitiligo at at Anandaban Hospital in Kathmandu with a lifelong history the level of the dermo-epidermal junction, parallel to the skin of skin problems causing mutilation of her face, hands and surface. This needle push, called needling (Savant SS. Surgical feet. Her mother reported that since the age of two years the therapy of vitiligo: current status. Indian J Dermatol Venereol Leprol patient’s urine had been pink. No other family members are 2005; 71: 307–10), drags epidermal cells including melano- affected. Examination of her skin revealed sclerodermatous-like cytes as a micro inoculation to produce multiple small popula- thickening on the face with beaking of the nose, microstomia tions of melanocytes in the vitiligo area, which is then and erythrodontia. There was scarring with deformity affect- exposed to increasing doses of narrow band UVB to cause ing the hands and feet. A marked hypertrichosis on the tem- melanogenesis and hence repigmentation of the area. Multiple ples with more lanugo-like hair on the cheeks was present. needle pushes are made through the edge one centimetre There was no hepatosplenomegaly. The full blood count was apart. All patients have needling once weekly and UVB 3 times normal. Her urine was pink and fluoresced under Wood’s a week with increasing doses. A study of this combination light. A diagnosis of congenital erythropoietic porphyria (CEP) treatment was done in 70 patients, of both sexes in different was made. Blood, urine and stool was transported to the U.K. age groups, from July 2005 to July 2006. A comparison was for biochemical and genomic analysis. The results showed also done with UVB alone (without needling) in some of markedly elevated urinary, faecal and erythrocyte porphyrins these patients for some patches. Inclusion and exclusion cri- with a plasma porphyrin peak of 618 nm confirming the clin- teria, and results criteria were defined and a study proforma ical diagnosis of CEP. A 25-year-old woman was seen with a with follow-up details was set up. Photos of all the patients history of skin blistering since the age of 5 days. She has scar- were taken at the start and then every third week. Biopsy of a ring leading to deformity of the nose and ears, and retraction few patients was performed to observe the effects of needling. of the upper lip revealing erythrodontia. She also has hypertri- This combination treatment has proved safe and very effective chosis, conjunctivitis and loss of tissue of the sclerae. Her against vitiligo as compared with UVB alone in all age groups. spleen was palpable 4 cm below the costal margin. She too All the patients had fast repigmentation. The best results were admitted to having red urine, only when asked directly. She on the face with more than 90% repigmentation in all age had a mild anaemia with thrombocytopenia. A diagnosis of groups in 8–12 weeks. Repigmentation is still good but probable CEP was made, also confirmed by a biochemical slower as we go away from the face towards peripheral parts diagnosis. CEP is the result of a mutation in the UROS gene of the skin. Repigmentation was specially noticed to start from which causes reduced activity of the enzyme uroporphyrino- the edge of the vitiligo patch (where needling is started) as gen III synthase that catalyses the cyclization of hydroxy- tiny black dots and further needling through these dots gradu- methylbilane (HMB) to uroporphyrinogen III. A homozygous ally encouraged repigmenting of the central areas. Repigmen- transition of T fi G at nucleotide 416 in exon 7 was identi- tation is also good over areas with grey hair with this fied in both these women. This mutation results in the neutral technique as needling uses surrounding epidermal cells rather polar amino acid leucine being substituted by the basic amino then the follicular cells. This further strengthened the idea of acid arginine. The leucine is highly conserved across species needling as a useful combination with UVB. including human, rat and yeast. This missense mutation has not been described before and appears to result in a moderate

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 132–138 134 British Photodermatology Group

PD-5 PD-6 Optimal timing for hand and foot photochemother- Impact of minimal erythema dose (MED) testing on apy following local immersion TL01 treatment: a prospective study S. Desai and S. Halpern S. Rajpara and M. White Medway Associated University Hospital, Gillingham, Kent, U.K. Dermatology Department, Aberdeen Royal Infirmary, NHS Grampian, Aber- Hand and foot photochemotherapy (PUVA) is a safe and deen, U.K. effective treatment for inflammatory dermatoses. Current prac- Minimal erythema dose (MED) testing is performed to deter- tice has been for immediate ultraviolet (UV)A irradiation after mine the starting dose for narrow-band UVB radiation (TL01) a 15 min soak in 3 mg L–1 8-methoxypsoralen (8-MOP) solu- treatment. The optimal site for doing MED testing is the back tion. Guidelines for topical PUVA from the British Photoder- (Dawe RS. Knowledge of body site variability in ultraviolet matology Group recommend a 30 min delay between the induced erythemal responses guides choice of site for pre- psoralen soak and irradiation, based on experimental evidence therapy minimal erythema dose testing (Letter). J Invest Dermatol of the time course of onset of photosensitisation using min- 2005; 124: 662). It has also been shown retrospectively that imal phototoxic dose (MPD) data on normal palmoplantar an approach based on skin type is at least as effective and safe skin. However, to date no published data exists on whether a as the MED based approach (Drummond A, Torley D, Jamie- delay in irradiation influences the efficacy of local immersion son CA et al. Narrowband ultraviolet B for psoriasis: ‘to MED PUVA in hand and foot dermatoses. This study aimed to or not to MED’, that is the question. Br J Dermatol 2003; 149 investigate whether the altered epidermis in diseased skin Suppl. 64: 2. Abstract). There has not been a prospective study necessitates a change in treatment timing. Patients received a assessing the impact of MED testing on TL01 treatment. This 15 min soak of affected extremities in 3 mg L–1 8-MOP solu- study examined the differences in the total number and dose tion and were randomized to have one side irradiated imme- of TL01 treatments required and the adverse effects based on diately and the contralateral side irradiated after a 30 min MED and non-MED (skin type) approaches. We enrolled 106 delay, using a Waldman hand and foot machine twice weekly consecutive subjects (49 males, 57 females) from July 2005 for up to 18 sessions. UVA dosage was commenced at until April 2006. MED testing was performed in all subjects 0Æ5Jcm–2 for palmar and plantar surfaces and 0Æ2Jcm–2 on the back with the Durham MED tester. The starting dose –2 (skin types I, II) or 0Æ3Jcm–2 (skin types III, IV) for dorsal was based on the skin type (0Æ14 J cm for skin types I and –2 skin, with increments of +0–40% depending on the develop- II, 0Æ20 J cm for skin types III and IV) in the first 51 sub- ment of erythema. This was a single blind study; clinical jects (group 1) and was 50% of the MED in a further 55 con- assessments were carried out by the same physician at baseline secutive subjects (group 2). Table 1 shows the results and again after 6, 12 and 18 treatments. The parameters (SD = standard deviation). The starting dose for TL01 was assessed were the extent of disease involvement, erythema, significantly higher in group 1 as compared with the starting hyperkeratosis, postulation and symptomatology (itch and dis- dose for group 2. There was no significant difference in total comfort) using a grading system and mapping of the affected dose, number of treatment sessions and doses causing ery- areas. Thirty-four patients were entered into the study, eight thema between two groups (Student unpaired t test). Instances men and 26 women with an age range of 20–74 years. Ten of erythema were reported as 38 and 57, respectively, for 2 patients had eczema, 13 had psoriasis (plaque or hyperkeratot- each group (not significant, v test). The dose range causing –2 ic), six had psoriasiform dermatitis and one had localized pus- erythema was 0Æ07–2Æ5Jcm in the MED group and 0Æ14– –2 tular psoriasis. Thirty patients had at least 10 treatment 2Æ5Jcm in the non-MED group. One episode of each of sessions and 24 completed the 18-session course. Small differ- grade 4 erythema and prickly heat were reported in the non- ences in lateralization were noted in six patients, with greater MED group. One episode of PLE was reported in the MED improvement seen on the side immediately irradiated and the group. In conclusion, although MED testing can be recom- side irradiated after delay in equal numbers. Much greater mended to exclude unexpected photosensitivity it did not improvement was noted in a further three patients on the make an impact on TL01 treatment. delayed irradiation side. Of these, two had hyperkeratotic psoriasis (one on acitretin) and one had hand dermatitis. This MED group Skin type group study has confirmed the efficacy of hand and foot PUVA. It Age range 18–84 years 16–73 years also suggests that for the majority of patients there is no add- No. of subjects (psoriasis) 41 35 itional benefit in delaying irradiation beyond the standard 15 Mean total dose of TL01 24Æ5Jcm–2 26Æ6Jcm–2 min immersion period. However as a 10% minority of the (SD 18Æ08) (SD 16Æ97) patients demonstrated a marked benefit from delaying irradi- Mean number of 25Æ7 (SD 10Æ75) 28Æ7 (SD 11Æ72) ation (of whom two of three had hyperkeratotic psoriasis), a treatments larger study is warranted to investigate the relationship between greater degrees of hyperkeratosis, i.e. stratum cor- neum thickness, and greater response to delayed irradiation.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 132–138 British Photodermatology Group 135

PD-7 PD-8 Cutaneous photoprotection by dietary tomato DNA damage in photodynamic therapy through product the production of reactive oxygen species parallels M. Rizwan, A. Harbottle,* S. Blanco, R. Watson, that of ionizing radiation and UVA M. Birch-Machin* and L. Rhodes A. Pye and A. Curnow Dermatological Sciences, School of Translational Medicine, University of Man- Peninsula Medical School, Truro, Cornwall, U.K. chester, Manchester, U.K. and *Dermatological Sciences, Institute of Cellular Aminolaevulinic acid photodynamic therapy (ALA-PDT) com- Medicine, University of Newcastle, Newcastle, U.K. bines a photosensitizer with light and tissue oxygen to pro- Lycopene is a carotenoid antioxidant found in tomatoes and duce reactive oxygen species (ROS) resulting in cellular other red fruits and vegetables, with anticarcinogenic proper- damage and ablation of tumour tissue. ALA-PDT has been ties. We have examined its potential photoprotective proper- shown to be an effective treatment for actinic keratosis and ties in (i) ultraviolet radiation (UVR)-induced skin erythema, superficial basal cell carcinoma (the methyl-ester of ALA by use of an erythema dose-response model, and (ii) UVR- (MAL) has now been granted regulatory approval in the U.K. induced mitochondrial DNA damage, a biological dosimeter and across Europe). Advantages over standard treatments for UVR exposure. A randomized controlled dietary supple- include better cosmetic outcome and the ability to treat mul- mentation trial was performed in 20 healthy females (mean tiple or extensive lesions. Cellular damage in this form of PDT age 33 yrs, range 21–47 yrs; all skin type I or II) who is mediated through excitation of the photosensitizer proto- received either 55 g tomato paste (containing 16 mg lyco- porphyrin IX and the subsequent production of ROS. These pene) in olive oil, or olive oil alone, daily for 12 weeks. At ROS are highly reactive chemical species that can react with the beginning and end of the supplementation period, a geo- and damage cellular components. ROS are also produced in metric series of 10 UVR doses were applied to buttock skin other treatment modalities. For example a significant portion using a broadband UVB lamp, and the erythemal response of the damage caused by ionizing radiation is mediated was assessed at 24 hours, both visually, as the minimal ery- through ROS formed by the ionization of endogenous oxygen thema dose (MED), and objectively through quantification species in the cell (as well as by direct ionization of cellular with a reflectance instrument. Biopsies were taken from unex- components). Similarly ultraviolet A (UVA) radiation (such as posed and 3 · MED UVR-exposed skin at the beginning and that used for PUVA) is also known to cause damage through end of supplementation, and were assessed for mitochondrial the production of ROS. The ability of ionizing radiation and DNA damage using the quantitative polymerase chain reaction UVA to cause DNA damage is well known, but the level of (qPCR) assay for analysis of UVR-induction of the Mt 3895 DNA damage generated in PDT is less well recognized. Cellu- deletion (Harbottle A, Birch-Machin MA. Real-time PCR analy- lar DNA damage was therefore measured in vitro using alkali sis of a 3895 bp mitochondrial DNA deletion in nonmelanom- single cell gel electrophoresis (comet assay) following expos- a skin cancer and its use as a quantitative marker for sunlight ure of human cultured cells to PDT, ionizing radiation or exposure in human skin. Br J Cancer 2006; 94: 1887–93). The UVA. The comet assay is able to detect both the single and median MEDs after taking the supplement in the tomato paste double strand breaks which can be induced by endogenously and control groups were 38 (range 23–53) vs. 27 (range formed ROS. This technique however cannot indicate whether 15–53) mJ cm–2, respectively, P =0Æ09. The mean D30 post this DNA damage has the potential to be mutagenic and this supplement, determined quantitatively by erythema dose warrants further detailed study. DNA damage was detected in response modelling, was 36Æ6 (SD 14Æ7) vs. 23Æ0 (SD 6Æ6) the cells after PDT (with either ALA or MAL). The damage mJ cm–2 in the tomato paste and olive oil groups, respectively, detected was related to both the level of photosensitizer pre- P =0Æ028. UVR-induced cutaneous mitochondrial DNA damage sent in the cells and the light dose administered. Similar levels was reduced after tomato paste, P <0Æ05, but showed no sig- of DNA damage were detected in the same cell type exposed nificant change after the control supplement. This pilot study to either ionizing radiation or UVA. This study shows that indicates protection by lycopene against acute UVR-induced PDT can cause DNA damage at levels similar to those observed skin effects. Further, it illustrates that the relatively crude with ionizing radiation and UVA irradiation. The implications assessment of the visual MED may fail to detect protection evi- for possible long-term genotoxicity with ALA-PDT should dent on dose-response modelling and tissue analysis. Small therefore be considered in the same way as those for ionizing degrees of UVR-protection obtained from lycopene-containing radiation and UVA. The risks from this genotoxic damage clin- food products could over a longer period play a significant ically are likely to be relatively small from individual PDT role in promotion of skin health. This project was supported treatments (which are designed to be cytotoxic) and are far by the British Broadcasting Corporation and appeared in the outweighed by the benefits of the treatment for actinic kerato- BBC-2 series ‘The Truth About Food’ 2007. sis and basal cell carcinoma. Where multiple treatments are delivered at lower noncytotoxic doses however the balance between outcome and risk may require more careful consid- eration.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 132–138 136 British Photodermatology Group

PD-9 sBCC had a total of 50 lesions and seven patients with BD had Audit of topical photodynamic therapy (PDT) 18 lesions. At 3 months 84% (42 of 50) of sBCC achieved T. Desai and S.B. Bittiner complete clearance, 10% (5) had partial clearance and 6% (3) Doncaster & Bassatlaw Hospitals Foundation NHS Trust, Doncaster, South failed to respond. This is comparable with the rates published Yorkshire, U.K. in previous studies (Kormeili T, Yamauchi PS, Lowe NJ. Top- Photodynamic therapy (PDT) is emerging as effective, safe ical photodynamic therapy in clinical dermatology. Br J Dermatol and acceptable treatment for superficial basal cell carcinoma 2004; 150: 1061–9). Similarly 95% (17 of 18) of BD lesions (sBCC), Bowen’s disease (BD) and actinic keratosis (AK). In completely resolved. Patients with AK had a poor response to December 2005, we started providing PDT to a selected group PDT, only 25% achieving complete clearance; 50% showed of patients who had either failed to respond to standard treat- partial clearance and 25% failed to respond. In retrospect we ment or had limitation of standard treatment because of the feel that our patients had hyperkeratotic and thick lesions size, site, and number of lesions, or co-existing medical con- compared with the nonhyperkeratotic lesions reported in most ditions. A clinic protocol was designed based on BAD guide- studies in the literature. PDT was also unsuccessful in curing lines and a database was created to aid audit and follow-up. one case each of extramammary Paget’s disease and solitary PDT was administered twice at weekly intervals for sBCC, BD giant porokeratosis. The evaluation of pain scores showed that and other conditions and once for AK. An audit was under- lesions on the lower limbs were more painful than those on taken to study adherence to protocol, the effectiveness of PDT the face, neck and scalp. Patients appreciated distraction ther- and adequacy of pain control measures. All patients who had apy, cold water sprays and cooling fan air to reduce pain dur- at least one follow-up visit at 3 months were included. Thirty- ing PDT. No change in pain scores were observed with local two patients (20 male and 12 female, age range 58–88 years) anaesthetic or analgesics when used. We have recommended had clinical diagnoses of sBCC (n=14), BD (n=7), AK (n=6), that AK patients should receive PDT twice at weekly intervals. sBCC+AK (n=3), extramammary Paget’s disease (n=1) and Long term follow-up is needed to assess the recurrence rate in solitary giant porokeratosis (n=1). Seventeen patients with our patients.

Posters

PD-10 sue. It was difficult to compare the fluorescent histology A pilot study to determine the effect of pressure specimens quantitatively in view of the variations in individual injection on the penetration of topical methyl tumours in each of the study groups but this pilot study aminolaevulinic acid (MAL) into nodular basal cell showed a definite trend towards an increase in the relative carcinoma (nBCC) of the skin tumour concentration of MAL in the nBCCs treated with Oxy- S. Campbell, S. Horton and A. Curnow jet enhanced delivery, compared with normal delivery. This Cornwall Dermatology Research, Truro Cornwall, U.K. small clinical pilot study showed promising results in the This pilot study looked at a way of enhancing penetration of enhancement of delivery of the topical photosensitizer MAL the photosensitizer into nodular basal cell carcinomas (nBCCs) using a novel pressure injection device. using an oxygen pressure injection device. Oxygen pressure injection (Oxy-jet) (Nora Bode, Hohenstein, Germany) is a method of driving compounds into skin using high pressure oxygen. The equipment has previously been shown to be effective in delivering cosmetic oils into the dermis. The study was an observer blinded pilot of a single application of topical PD-11 methyl aminolaevulinic acid (MAL) to nBCCs, with or without Solar capillaritis as a cause of solar purpura: a case the use of the Oxy-jet. Six patients were recruited into each of report the five study groups in strict order and following application D. Sandhu, A. Waters, C. Green* and J. Ferguson of MAL, the basal cell carcinomas were excised using a stand- Photobiology Unit, Ninewells Hospital and Medical School, Dundee, U.K. and ard surgical approach at 0, 30, 60, 120 and 180 minutes. The *Department of Dermatology, Ninewells Hospital and Medical School, Dundee, excised specimens were kept in the dark and examined by U.K. conventional histology to assess thickness of tumour and Solar purpura is a rare phenomenon, with only eight previ- tumour type and in addition sections of the tumour were ously described cases, in which purpuric lesions appear examined with fluorescence microscopy to determine the acutely following sun exposure. We now report on a further depth of penetration of the MAL and also by intensity of fluor- case that we recently investigated. A 51-year-old lady was escence, the relative concentration of the compounds in the tis- referred to our department with a 20-year history of an

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 132–138 British Photodermatology Group 137 asymptomatic eruption appearing on her legs after sun expos- sis factor a (TNFa;25ngmL–1) and hydrogen peroxide ure. It appeared annually 2–4 days after the first prolonged (1, 10, 100, or 1000 lmol L–1). Cells were assessed for type sun exposure of the year, and typically persisted from June to of death by using annexin V binding and fluorescence micro- September. The morphology was of grouped nonpalpable scopy after 45 h. The data showed that there was a statistically petechiae confined to the photoexposed skin between her significant increase in the percentage of apoptosis compared knees and ankles. It did not occur at other sites, even though with the nonexposed control groups (P <0Æ05; Student’s t-test they had received similar sun exposure. There was no relevant and two-way ANOVA, P <0Æ01 respectively). After setting up a past medical history, and she was not taking any medication reliable and consistent PDT system, fibroblasts were exposed at the onset of her condition. Phototesting was performed in to 0Æ5 mmol L–1 of ALA for 6 h and exposed to a red light duplicate on her back and lower legs. Monochromator testing (630 ± 15 nm; 100 J cm–2; Paterson) for 5 min. The cells of both sites produced normal responses at all tested wave- were assessed for cell death 16 h after irradiation. The PDT- lengths (305–430 nm). Provocation testing was performed exposed population demonstrated a statistically significant with narrow-band UVB, broad-band UVA, visible, and infra- increase in percentage of apoptosis compared with the red sources (4 · 4 cm daily exposures over 3 days). UVA non-exposed control groups (P <0Æ01; Student’s t-test). To provocation (60 J cm-2 total) on the lower leg evoked a non- investigate which ROS are being produced during ALA-PDT, palpable petechial eruption which became apparent 72 hours electron paramagnetic resonance spectrometry (EPR) has been after the first exposure. Biopsy of the eruption showed used. EPR is one of the most direct and defined methods of features of a lymphocytic capillaritis, with negative direct measuring free radicals. To date the superoxide adduct has immunofluorescence. Equivalent UVA testing on the patient’s been trapped using the long established method of reacting back did not produce an abnormal response. Provocation test- xanthine with xanthine oxidase. This in vitro cell model will be ing with narrow band UVB, visible, and infrared sources failed further employed to explore the effects of pro- and antioxi- to produce any abnormal responses at either test site. The full dant agents. blood count, urea and electrolytes, liver function tests, coagu- lation screen, C reactive protein, plasma viscosity, antinuclear antibodies, extractable nuclear antibodies, and plasma porphy- ria scan were all within normal limits. We diagnosed the patient as having a photo-induced capillaritis, which we have PD-13 termed ‘solar capillaritis’. This is a hitherto unrecognised con- A retrospective study to assess oral versus topical dition, but we believe it may account for almost half of the psoralen ultraviolet A photochemotherapy for the previously published cases of solar purpura. The remaining treatment of hand and foot eczema published cases differ in a number of ways, and it therefore A. Mitra, A. Yung, D. Turner, F. Elliott* and V. Goul- appears that solar purpura is a feature of several distinct condi- den tions, rather than a single entity. Leeds General Infirmary, Leeds, U.K. and *St James’s University Hospital, Leeds, U.K. Oral and topical psoralen photochemotherapy (PUVA) are effective and generally safe treatments for hand and foot eczema. Studies comparing oral and topical PUVA for localized PD-12 eczema are scarce. The two modalities have previously been Mechanisms of cell death induced by dermatological found to be equally effective for chronic hand and foot der- protoporphyrin IX-induced photodynamic therapy matoses (Hawk JLM, Grice PL. The efficacy of localized PUVA Y. Dogra, A. Curnow and P. Winyard therapy for chronic hand and foot dermatoses. Clin Exp Dermatol Peninsula Medical School, Universities of Exeter and Plymouth, Exeter, U.K. 1994; 19: 479–82). A retrospective study of patients treated Aminolaevulinic acid-induced photodynamic therapy (ALA- with PUVA for hand and foot eczema was carried out between PDT) is a sophisticated and highly selective way to ablate tis- January 1999 and January 2005 in the U.K. Data were collec- sue. There are many applications of ALA-PDT, but this project ted from case notes and a phototherapy database. The out- will be focusing on the use of the treatment for nonmelanoma come was assessed under the supervision of the consultant skin cancer, in particular, basal cell carcinoma (BCC). BCC dermatologist responsible for the phototherapy service and forms in basal cells deep in the epidermis and is the most categorized as clear, minimal residual activity (MRA), good common type of nonmelanoma skin cancer, accounting for improvement or poor outcome (moderate, mild or no approximately 75% of the total. The current study investigates improvement). The aim was to assess any difference in out- the molecular mechanisms of ALA-PDT induced apoptosis in come between oral and topical PUVA. A total of 110 courses cells (fibroblasts, keratinocytes and squamous carcinoma cells) of PUVA were carried out in 104 patients. Fifty-five courses of assessing the amount and type of cell death occurring and also treatment were carried out using oral 8-methoxypsoralen the reactive oxygen species (ROS) being produced. As positive (8-MOP) and 55 with topical 8-MOP soaks. The sex distribution controls in experiments when investigating the effect of ALA- and mean age of the patients was similar in both groups. PDT and cell death, fibroblasts were exposed to tumour necro- There was no significant difference in the mean duration of

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 132–138 138 British Photodermatology Group eczema (41 months, oral group and 40 months, topical pared with 31% of the topical group. The oral group had a group; P =0Æ9), Fitzpatrick skin type (P =0Æ8) or distribution significantly better outcome than the topical group (v2(2) of pompholyx or hyperkeratotic eczema (P =0Æ3) between 14Æ4, P =0Æ001). In the topical group, 54% had an allergic or the two groups. In the oral group, 54% had both hand and irritant component to their eczema compared with 31% of the foot eczema compared with 22% of the topical group. No sig- oral group. Further analysis showed that this did not account nificant difference was seen in the use of concurrent topical or for the difference in outcome between the oral and topical oral medications or previous treatments. The median number group. More side-effects were seen in the oral group (47%) of treatments was similar (19 treatments, oral group and 20 than in the topical group (33%) but this was not significant treatments, topical group). No significant difference was seen (P =0Æ1). In the oral group, 24% changed from 8-MOP to 5- in the mean cumulative dose (111 J cm-2, oral group and 98 MOP during treatment, 85% of which was due to nausea. This ) Jcm 2, topical group; P =0Æ4). Fifty-four per cent of the oral study suggests that oral PUVA is more effective than topical group cleared compared with 21% of the topical group; 29% PUVA for hand and foot eczema but is associated with more of the oral group achieved MRA or good improvement com- side-effects.

2007 The Authors Journal Compilation 2007 British Association of Dermatologists • British Journal of Dermatology (2007) 157 (Suppl. 1); 132–138