Bristol Cup Posters: Summaries of Posters

P-1 P-2 Nurse-led diphencyprone treatment clinic: A case of SAPHO syndrome: did isotretinoin development of the role of the DPC treatment trigger it? treatment nurse L. Paul and J.A. Newton Bishop E. Walsh and S. MacDonald Hull St James’s University Hospital, Leeds, U.K. Mid Yorkshire Hospitals NHS Trust, Pontefract, U.K. SAPHO syndrome is characterized by any combination of A registered nurse trained in the use and application of diph- synovitis, , pustulosis, and (Hayem encyprone (DPC) runs a weekly DPC treatment clinic. She has G, Bouchaud-Chabot A, Benali K et al. SAPHO syndrome: a in-house training assessed by a multiple choice questionnaire long term follow up study of 120 cases. Semin Arthritis Rheum and quarterly formal reviews in the form of ‘observed prac- 1999; 29: 159–71). We describe a case that may have been tice’ for 12 months after completing training. The study triggered by isotretinoin therapy. A 14-year-old boy had acne describes her training, her management of a patient starting vulgaris affecting his face, chest and back that had deteriorated DPC treatment, giving details of pretreatment investigations, recently. Scarring was noted. He was systemically well, in par- consent to treatment, treatment documentation, practical ticular had no fevers or joint pains. His previous medical his- details relating to the handling, use and storage of DPC, and tory included learning difficulties and well-controlled epilepsy. advice given to patients. She also describes the setting up and He was commenced on isotretinoin 0Æ3 mg kg–1 daily for 2 ongoing development of a monthly Self Help Group and the weeks before increasing to 0Æ6 mg kg–1 daily. His GP added results of three audits evaluating different aspects of the ser- in trimethoprim 300 mg twice daily and his skin began to vice. Audit one: The patient’s perspective on the value of the improve. However after 4 weeks at the higher dose of isotre- Self Help Group. This audit confirmed that patients derive tinoin, he developed bony pain, an abnormal gait and consti- practical and psychological benefit from attending the Self pation. He was admitted by the general surgeons. An MRI Help Group. Audit two: Sensitization of children (16 years scan revealed spondylolisthesis at L5/S1, with abnormal sig- and under) to DPC. The aim was to identify the number of nals at C7, T10/T11 and both sacral ala, suggestive of an infil- children who required more than one application of DPC to trative process. Blood tests, bone marrow culture and bone become sensitized. Nineteen children were included; their biopsy showed no infective or neoplastic features. In retro- mean age was 11Æ6 years. Nine children had , spect, a diagnosis of aseptic osteitis was made suggesting the one , three alopecia sub-totalis, four alopecia SAPHO syndrome. In this patient the isotretinoin was stopped sub-universalis and two . Of these, four and his bony symptoms resolved spontaneously. His acne vul- required two applications and one required three applications garis has since been controlled using trimethoprim and low- to become sensitized. Audit three: Comparison of the treat- dose dapsone, with no recurrence of bony symptoms. The ment outcome for children requiring more than one applica- SAPHO syndrome is characterized by a combination of skin tion to become sensitized with those who were sensitized and bony pathology. The skin disease is either acne of the ful- after one application. The same cohort as for Audit two was minans or conglobata type, or suppurativa, or studied. Of the 10 requiring more than one application for pustulosis including palmoplantar psoriasis or pustular psoria- sensitization, three with alopecia areata and one with alopecia sis. The characteristic bone feature is aseptic osteitis, most totalis achieved complete regrowth; two with alopecia sub- commonly affecting the anterior chest wall, sacroiliac joints universalis achieved almost complete regrowth, two with alo- and vertebrae (Hayem G et al. 1999). A previous case has been pecia areata had no regrowth, one with alopecia sub-univer- reported in which isotretinoin appeared to trigger SAPHO syn- salis discontinued treatment and one with alopecia universalis drome (Chua SL, Ravenscroft J. : part of the moved to another Trust. Of those sensitized after one applica- spectrum of SAPHO. Br J Dermatol 2007; 156: 1408 Abstract). tion, four with alopecia areata completely regrew their hair, Reintroduction of the isotretinoin reproduced the same symp- one each with alopecia areata, alopecia sub-totalis and alopecia toms in that patient. In our case, the timing of the initiation universalis and two with alopecia sub-universalis did not of isotretinoin and simultaneous development of symptoms, regrow. There was no statistical difference between the two as well as resolution of the condition on withdrawal of groups. the drug, would suggest that the bony aspect of the SAPHO syndrome was triggered by the isotretinoin.

Ó 2008 The Authors 24 Journal Compilation Ó 2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 25

P-3 P-4 Do we require age-specific reference ranges for The management of primary cicatricial alopecias androgen values when assessing females with M. Harries, R. Sinclair,* S. MacDonald Hull, cutaneous disorders associated with D. Whiting,à C.E.M. Griffiths and R. Paus§ hyperandrogenism? The Dermatology Centre, The University of Manchester, Manchester, U.K.; I. Ali, T. Jones,* N. Meston,* B. Shine* and *Department of Dermatology, University of Melbourne, Melbourne, Austra- F.T. Wojnarowska lia; Department of Dermatology, Pontefract General Infirmary, Pontefract, John Radcliff Hospital, Oxford, U.K. and *Department of Dermatology, U.K.; àBaylor Hair Research and Treatment Centre, Dallas, U.S.A. and Churchill Hospital, Oxford, U.K. §Department of Dermatology, University of Lubeck, Lubeck, Germany Androgen assays are important in the investigation of viriliza- The primary cicatricial alopecias (PCA; scarring alopecias; per- tion, and alopecia in females. The latter two of these manent alopecias) are a diverse group of inflammatory hair conditions have a greater prevalence in the postmenopausal disorders of unknown aetiology that result in irreversible hair age group. Androgen levels can change with increasing age loss. They are characterized clinically by permanent loss of vis- and menopausal status (Longcope C. Hormone dynamics at ible follicular ostia and pathologically by replacement of follic- the menopause. Ann N Y Acad Sci 1990; 592: 21–30). The exact ular structures with fibrous tissue. In PCA the hair follicle is nature of these changes is a controversial topic; there have the main target of the disease process whereas in secondary been a limited number of studies with varying methodology cicatricial alopecias the follicular damage occurs indirectly and follow-up. Adrenal androgen levels peak in the mid 20s from events occurring outside the follicular unit (e.g. trauma, and thereafter decline in a linear manner which appears to be infection, etc.). The management of PCA is challenging. Poor independent of the menopause. Testosterone levels have been correlation between clinical and pathological features has shown to decline just prior to the menopause and then rise in resulted in problems with disease classification, with incom- the late menopause. Studies of sex hormone binding globulin plete or inaccurate disease definition and inconsistent use of (SHBG) levels in the ageing female have variable results terminology in the medical literature. Monitoring disease (Longcope C 1990). Reference intervals provided by most lab- activity is also notoriously difficult, particularly in conditions oratories for androgens do not include specific data for post- with minimal visible inflammation, e.g. pseudopelade of menopausal status. This could lead to misinterpretation of Brocq. To complicate matters, no fully satisfactory, evidence- androgen values. Age-specific reference ranges may provide based regimens are available for the treatment of defined scar- greater diagnostic accuracy. The objective of this study was to ring alopecias. In 2001 the North American Hair Research determine population specific reference intervals and evaluate Society produced a working classification of PCA based on the changes related to age for dihydroepiandrosterone (DHEAS), predominant inflammatory infiltrate found on scalp biopsy. androstenedione, testosterone, SHBG and free androgen index Differentiation of recognized clinical conditions, within each (FAI). Serum was collected from 182 postmenopausal females histological subgroup, could then be made, based on the clini- aged 45 or over using a standard technique and stored at cal features expressed. The main aims of treatment are to )70ºC until analysis. The results were divided into four age reduce symptoms and, most importantly, to slow or stop pro- groups, 45–54, 55–64, 65–74 and >74 years. A Kruskal–Wal- gression of the scarring process. We present an evidence-based lis test was performed to assess the differences between andro- summary of treatment options for PCA and propose a thera- gen and SHBG values between the different age groups. peutic hierarchy based on a broad appraisal of the reported Androstenedione (P < 0Æ05) and DHEAS significantly efficacy and safety of each agent. Studies were identified from decreased with increasing age (P < 0Æ001). There was no sig- Medline searches, manual searches and from personal experi- nificant difference in testosterone, SHBG or FAI with age. ence. The quality of evidence for each treatment will be dis- These findings would suggest that in order to develop greater played as follows: (A) randomized controlled trial (RCT); (B) diagnostic accuracy when interpreting androgen assay results study with > 20 participants; (C) study with < 20 partici- there should be separate reference ranges provided for DHEAS pants; (D) series of > 5 cases; and (E) case report or expert and androstenedione with age groups. Previous studies have opinion. In general, the lymphocyte-predominant subgroup of shown a perimenopausal decline in testosterone suggesting PCA are treated with immunosuppressants and the neutrophil- testosterone values should be divided by menopausal status. predominant subgroup with antimicrobials or dapsone. Using The significant variance with general reference ranges across a chronic cutaneous lupus erythematosus as an example, the rec- wider age group may result in misinterpretation of results, ommended first-line therapies are potent topical corticoster- e.g. a raised DHEAS level in an older female could be deemed oids (A) or intralesional triamcinolone acetonide (C); second- to be within the normal range. More work is required to clar- line therapies are antimalarials (A) or oral retinoids (A) and ify this issue and to correlate assay results with clinical find- third-line therapies are thalidomide (B), topical immunomod- ings in medical conditions associated with hyperandrogenism. ulators (A), oral vitamin E (B), oral gold (B), dapsone (B) or clofazimine (B). An accessible, practical and hierarchical model for management of PCA has been developed for use in clinical practice.

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 26 Bristol Cup Posters

P-5 tent erythema (erythematotelangiectatic , ETTR), and An audit of the management of hidradenitis that dilated vessels subsequently leak serum resulting in an in- suppurativa: do we ever succeed? flammatory reaction of papules and pustules (papulopustular C. Devereux and K. Armstrong rosacea, PPR). To evaluate this theory we determined the Royal Victoria Hospital, Belfast, U.K. prevalence and demographic characteristics of ETTR and PPR A retrospective audit was conducted in all patients attending a and their relationship to photodamage. A two-phase, cross- large teaching hospital with a diagnosis of hidradenitis suppu- sectional epidemiological study of 1000 individuals was car- rativa (HS) in 2005. A total of 32 patients were identified ried out determine the prevalence of PPR and ETTR and their using a computerized dermatology outpatient coding system. relationship to photodamage. Five hundred indoor workers We examined 31 charts – one chart was unobtainable. Female randomly sampled from a hospital workforce (predicted low patients accounted for 26 of the patients, in agreement with UV exposure) and 500 randomly sampled inhabitants of the most sizeable studies showing a preponderance of females. The Aran islands (predicted high UV exposure) were assessed. A age range of those affected was 18–58 years with an average structured questionnaire was administered to each individual age of 34 years. The duration of disease ranged from 4 months and their facial skin examined for evidence of rosacea and to 30 years, with an average of 5Æ4 years. Axillary lesions were photodamage. Variables recorded on each individual included the most commonly noted (25), followed by perineal lesions age, occupational and recreational outdoor exposure, (22), inframammary lesions (12) and with neck involvement frequency of flushing, presence of facial erythema and telangi- rare (1). It was anticipated that patients treated for HS would ectasia, and photodamage score using the Griffiths photonu- have been treated with many pharmacological agents. In real- meric scale (Griffiths CEM, Wang TS, Hamilton TA et al. ity, 17 of the patients audited had been treated with only one A photonumeric scale for the assessment of cutaneous photo- agent, four with two agents, three with three and four agents, damage. Arch Dermatol 1992; 128: 347–51). Using accepted cri- with seven as the greatest number of therapeutic strategies used teria (Wilkin J, Dahl M, Detmar M et al. Standard classification in one single patient. The overall remission rate was 52% (16 of rosacea: report of the National Rosacea Society Expert Com- of 31). Nine of the patients failed to return for follow-up after mittee on the classification and staging of rosacea. J Am Acad the initiation of therapy, therefore the outcome of therapy is Dermatol 2002; 46: 584–7), 27 individuals had PPR (prevalence unknown. After their first visit five were discharged with a 2Æ7%). The criteria for ETTR were fulfilled by 112 individuals treatment plan for mild severity of the disease. One patient’s (prevalence 11Æ2%). In the PPR group 12 were female; in the disease failed to improve despite multiple therapies. The most ETTR group 73 were female. The mean age was 48 years in commonly prescribed drugs were tetracyclines (11 of 31 had the PPR group and 47 years in the ETTR group. There was no been treated at one point with tetracyclines), followed by clin- significant difference in the prevalence of PPR in the hospital damycin and rifampicin combination (10), doxycycline (10), workers (low UV exposure) and the Aran islanders (high UV erythromycin (7), minocycline (5), isotretinoin (4), dapsone exposure). There was a significantly greater proportion with (1), (1) and metronidazole (1). Two were treated ETTR in the high UV exposure Aran islanders (16Æ3%) than in with surgical intervention. No patients were treated with bio- hospital workers (6Æ8%) (P < 0Æ0001), which persisted after logical agents. The highest remission rates were in response to adjustment for age. Of individuals with PPR, 15% had had doxycycline (50%), tetracycline (36%), clindamycin and rif- photodamage scores of 6 or greater. There was no significant ampicin combination therapy (30%), isotretinoin (25%), mi- association between photodamage and PPR. In the ETTR group nocycline (20%) and erythromycin (14%). No improvement 28Æ6% had a photodamage score of 6 or greater. The mean was noted in response to dapsone or ciclosporin therapy. Of photodamage score in those with ETTR was significantly interest, the clindamycin and rifampicin combination therapy greater than in those without (4Æ34 vs. 3Æ87; P = 0Æ01). This was not as beneficial in this cohort of patients as in those pre- persisted after adjustment for age. The differences in UV viously reported, of whom 57% obtained remission (Mendonc¸a exposure between individuals with ETTR and PPR, despite CO, Griffiths CEM. Clindamycin and rifampicin combination similar mean ages in both groups, does not support the therapy for . Br J Dermatol 2006; 154: theory that rosacea is a disease that progresses from ETTR 977–8). to PPR.

P-6 Prevalence and pathogenesis of rosacea M.A. McAleer, P. Fitzpatrick and F.C. Powell The Regional Centre of Dermatology, Mater Misericordiae University Hospital, Dublin, Ireland The aetiology of rosacea is unknown. It has been suggested that frequent facial flushing results in telangiectasia and persis-

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 27

P-7 P-8 Antibiotics used in the treatment of rosacea affect Epidermolysis bullosa simplex with mottled proliferation and lipid production in SZ95 cells pigmentation: clinical and molecular N. Lacey, K. Kavanagh,* H. Seltmann, C. Zouboulis confirmation in five subjects and F.C. Powellà T. Wong, A. Terron-Kwiatkowski,* Clinical Research Unit, University College Dublin and Mater Misercordiae F.T. Wojnarowska, D.U. Baty,* W.H.I. McLean,à University Hospital, Dublin, Ireland; *Department of Biology, National J.A. McGrath, A.E. Martinez§ and J.E. Mellerio University of Ireland, Maynooth, Co. Kildare, Ireland; Departments of St John’s Institute of Dermatology, London, U.K.; *Tayside University Dermatology and Immunology, Dessau Medical Center, Dessau, Germany and Hospitals NHS Trust, Dundee, U.K.; University of Oxford, Oxford, U.K.; àRegional Centre of Dermatology, Mater Misercordiae University Hospital, àUniversity of Dundee, Dundee, U.K. and §Great Ormond Street Hospital for Dublin, Ireland Children NHS Trust, London, U.K. The aetiology of papulopustular rosacea (PPR) is unknown. Epidermolysis bullosa simplex with mottled pigmentation Selective antibiotics clear inflammatory lesions but their mode (EBS-MP) is a rare autosomal dominant disease characterized of action is unknown (Powell FC. Clinical practice: Rosacea. by trauma-induced blistering, distinctive mottled pigmentation [Review]. N Engl J Med 2005; 325: 793–803). Demodex mite of the trunk and limbs, and punctate or focal palmoplantar numbers are increased in PPR patients and they may play a keratoderma (PPK). The majority of reported cases of EBS-MP pathogenic role in this disorder (Lacey N, Delaney S, Kava- are caused by a point mutation in the nonhelical head domain nagh K, Powell FC. Mite-related bacterial antigens stimulate of the keratin 5 gene (KRT5) leading to a proline to leucine inflammatory cells in rosacea. Br J Dermatol 2007; 157: 474– substitution, P25L. We present five patients with EBS and mot- 81). They feed on sebum produced by sebaceous glands. The tled pigmentation. Patient 1 is a 3-year-old boy with mild effect of three antibiotics, used in the treatment of rosacea blistering of the extremities with mottled pigmentation and (tetracycline, doxycycline and metronidazole), on cell prolifer- coarse hair; mutation analysis revealed the common KRT5 ation and lipid synthesis in an immortalized human sebocyte mutation, P25L. Patient 2 is a 10-year-old girl with mild skin cell line (SZ95) was analysed. Toxicity assays with the SZ95 blistering, mottled pigmentation of the trunk, nail dystrophy cell line were performed using a spectrum of concentrations and wiry, dry hair; mutation analysis also revealed the KRT5 of the chosen antibiotics to assess their effect on cell prolifera- mutation, P25L. Patient 3 is an 8-year-old boy diagnosed in tion. Alongside these tests, Nile red assays using spectrofluo- infancy with Dowling–Meara EBS. Skin fragility ameliorated rometer analysis, were carried out to evaluate the possible over time and he subsequently developed mottled pigmenta- alteration of lipid synthesis in the cells, when exposed to each tion on the trunk, punctate PPK, nail dystrophy and coarse, antibiotic concentration. Oil red staining was used to visualize wiry hair; mutation analysis showed a novel mutation A468P lipids in the sebocyte cells. SZ95 cells treated with tetracycline in KRT5. Patient 4 is a 16-year-old boy diagnosed with Dow- and doxycycline exhibited similar traits; a correlation was ling–Meara EBS as a neonate but with skin blistering now observed between reduced cell proliferation and reduced lipid mainly on the feet, dystrophic nails and coarse hair. Mottled synthesis. Both of these drugs inhibited proliferation at higher pigmentation of the trunk and lower limbs developed in ado- concentrations and as the concentration decreased, cell prolif- lescence, genetic analysis demonstrated the common Dow- eration increased. However, metronidazole-treated cells, ling–Meara EBS missense mutation, R125C in KRT14. Patient 5 showed no relationship between cell proliferation and lipid is a 24-year-old man with Dowling–Meara EBS diagnosed in synthesis. At one concentration (12Æ5 lg mL–1), metronida- childhood. Skin fragility has improved with age but mottled zole was shown to decrease cell proliferation by almost 50% pigmentation of the trunk has been observed, with dystrophic but lipids were found to be increased by over 45% in these nails and punctate PPK; mutation analysis revealed a novel cells in comparison to the control. This result was also sub- mutation in KRT14, L122R. The precise mechanisms by which stantiated by oil red staining. Antibiotic treatment of PPR may these mutations cause mottled pigmentation remain unclear, have an effect on sebaceous cells in vivo. This could alter the although perturbation of normal melanosome transportation Demodex mites’ food source and their follicular niche. and distribution, have been proposed. Although mottled Funding for this project was generously supplied by the pigmentation has been described in Dowling–Meara EBS, the National Rosacea Society of America. inter- and intrafamilial variability of this clinical sign is not well understood and appears unrelated to recent blistering epi- sodes. It is not clear whether these patients should be regarded as having EBS-MP, or simply mottled pigmentation as a feature of their Dowling–Meara EBS. The association of coarse, wiry hair has not previously been reported with EBS-MP, nor with any other form of EB. Delineation of mutations in our cases may help to provide new insights into the molecular mecha- nisms underlying pigmentation and the role of basal keratins in this process, in addition to genotype–phenotype correlation in this form of EBS.

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 28 Bristol Cup Posters

P-9 P-10 The use of rituximab as a single therapy for the Generalized vitiligo following sibling allogeneic treatment of severe recalcitrant pemphigus stem cell transplant S.I. Goolamali, G. Mufti, S. Warnakulasuriya and T. Joseph, S. Jain and C.J.M. Stephens A.W.P. du Vivier Poole Hospital NHS Trust, Poole, U.K. King’s College Hospital, London, U.K. Vitiligo is a condition with progressive destruction of melano- Pemphigus vulgaris (PV) is a rare potentially life-threatening cytes which is believed to be of autoimmune origin. We autoimmune disease characterized by cutaneous and/or muco- report a case of generalized vitiligo in a 25-year-old patient sal blistering. Current treatment is largely based on systemic that developed 18 months after bone marrow transplant for immunosuppression, with the use of corticosteroids combined acute lymphoblastic leukaemia (ALL). This 25-year-old gentle- with steroid-sparing agents such as azathioprine (AZA), meth- man was diagnosed with ALL in November 2004. On 13 June otrexate, ciclosporin and mycophenolate mofetil (MMF). A 2005 he underwent sibling allogeneic bone marrow transplant 57-year-old Asian gentleman was referred to the Dermatology following chemotherapy. There was a history of childhood Department from Oral Medicine with a 10-month history of eczema. Immediately following the transplant he had grade 1 buccal mucosal and palatal ulceration and erosions on his face, skin graft versus host disease (GVHD) affecting his ears and chest and back. Oral and cutaneous biopsies confirmed the face which was controlled with topical betamethasone 0.1%. diagnosis of pemphigus vulgaris. Direct immunofluorescence He was maintained on oral steroid and ciclosporin. He later (IMF) showed epidermal intercellular staining with IgG and developed chronic gastrointestinal and pulmonary GVHD C3. Indirect IMF demonstrated intercellular IgG antibodies to a which started 4 months and 9 months post-transplant, respec- titre of 1 : 100. He had been on prednisolone 60 mg daily tively. Skin changes at this time were minimal with eczema followed by a reducing course for 6 months without improve- on his back and lichenoid changes on his lips. He then devel- ment. The addition of AZA had resulted in secondary hepatitis oped rapidly progressive depigmentation of the skin without and it was discontinued. Prednisolone was restarted at a dose sclerosis 18 months post-transplant. On examination he had of 60 mg daily but despite the addition of MMF (1Æ5 g twice extensive areas of depigmentation with no sclerosis. He also daily) and ciclosporin 200 mg twice daily (5 mg kg–1), the gave a history of alopecia areata 14 months post-transplant lesions persisted. He was therefore considered suitable for rit- which resolved spontaneously. There was no family history of uximab therapy. MMF and ciclosporin were stopped and the vitiligo or other autoimmune diseases. A skin biopsy was done prednisolone gradually reduced. Infusions were administered which excluded cutaneous GVHD. He remains in complete weekly for 8 weeks (eight courses) and then fortnightly (two clinical remission for his ALL but his vitiligo is progressing. courses) with a good clinical response. He has subsequently Vitiligo has been reported as a rare complication of bone mar- received them monthly (one course) and 6-weekly (four row transplant (Cho SB, Roh MR, Chung KY et al. Generalized courses) and is now in clinical remission. The treatment was vitiligo after allogeneic bone marrow transplantation. Acta Derm well tolerated. Rituximab is an anti-CD20 chimeric monoclo- Venereol 2005; 85: 82–3; Au WY, Yeung CK, Chan HH, Lie nal antibody which has been shown to be effective in various AK. Generalized vitiligo after lymphocyte infusion for relapsed autoimmune diseases, including recently pemphigus resistant leukaemia. Br J Dermatol 2001; 145: 1015–17). In the absence to conventional therapy with corticosteroids and immunosup- of vitiligo in the donor, the autoimmune cascade occurring in pressive drugs. The combination of multiple cycles of ritux- GVHD may have led to the development of vitiligo in our imab and intravenous immune globulins was reported to be case. As previously reported, the vitiligo is continuing to pro- effective in 11 patients with pemphigus from a single centre gress even though his GVHD is under control. (Ahmed AR, Spigelman Z, Cavacini LA, Posner MR. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulins. N Engl J Med 2006; 355: 1772–9). How- ever, a recent multicentre series of 21 patients with severe refractory pemphigus advocated the use of a single cycle of P-11 rituximab (four weekly infusions) without intravenous Multiple giant keratoacanthomas on the lower legs immune globulins (as in our case), with 18 of 21 patients T. Joseph, S. Jain and C.J.M. Stephens achieving complete remission at 3 months (Joly P, Mouquet Poole Hospital NHS Trust, Poole, U.K. H, Roujeau J-C et al. A single cycle of rituximab for the treat- We report an unusual case of sudden onset of multiple giant ment of severe pemphigus. N Engl J Med 2007; 357: 545–52). keratoacanthomas on the lower legs. The only relevant history Due to potentially severe side-effects, its use should be regarding a possible aetiology in our patient is that of arsenic restricted to the most severe types of the disease, but it is very intake in the form of Parish’s food syrup as a child. In the effective in such difficult cases. absence of the classical skin signs of arsenic intake such as arsenic keratoses or nonmelanoma skin cancers, the relevance of arsenic in the precipitation of keratoacanthomas in our patient is debatable. A fit 78-year-old lady presented to derma- tology with a 5-week history of multiple large painful nodules

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 29 on both lower legs. They measured up to 3 · 3 cm and had extending from the eyes onto the chin. The margins of the crusted and keratotic centres. There was minimal sun damage rash were palpable with some minimal epidermal change. of the surrounding skin and no history of skin cancer. These There was no evidence of oral involvement. An incisional bi- lesions were debulked by curettage and cautery, and histology opsy showed mild epidermal hyperkeratosis with dyskeratotic showed the typical features of keratoacanthoma. She was cells in the epidermis and basal cell vacuolation. Numerous simultaneously started on acitretin 10 mg daily increasing to melanophages and cytoid bodies were seen with a band-like 25 mg. However, this did not prevent new lesions from perivascular infiltrate of lymphocytes and histiocytes in keep- appearing and she had to stop at 4 months because of side- ing with the clinical diagnosis of KLC. To date there have been effects. Some of her lesions responded partially to intralesional 14 reported cases of KLC in the paediatric population and steroids. She is continuing to develop new lesions on her approximately 50 reported adult cases. In adults it typically lower legs and we are considering other treatment options presents with keratotic papules on the limbs and buttocks with such as radiotherapy or cyclophosphamide. The aetiology of an erythematous reticulate mediofacial eruption. Associated keratoacanthoma is poorly understood. Epidemiological data mucosal involvement, palmoplantar changes and nail dystro- suggests that the incidence is related to sun exposure. Contact phy may also be seen. In contrast, in children facial lesions with tar and mineral oil has also been shown to cause an are the first to appear. Although our case is unusual in that increased incidence. There have been cases reported following the patient exhibits predominantly facial lesions, similar cases injury and in association with internal malignancy. Of the have been previously reported in the literature (Arata J, Seno multiple types, the most well recognized is the multiple self- A, Tada J et al. Peculiar facial erythematosquamous lesions in healing keratoacanthomas of Ferguson-Smith which arise in two siblings with cyclical summer improvement and winter sun-exposed areas, involute spontaneously and have an auto- relapse: a variant of keratosis lichenoides chronica? J Am Acad somal dominant inheritance (Wee SA. Multiple eruptive kera- Dermatol 1993; 28: 870–3). Treatment is difficult, although a toacanthomas, de novo. Dermatol Online J 2004; 10: 19). number of cases in this age group have noted spontaneous Grzybowski described widely disseminated small papular le- improvement in the summer months leading to subsequent sions, some of them typical keratoacanthomas affecting the treatment in other paediatric patients with PUVA. Topical cal- face, the trunk and proximal limbs (Hass N, Schadendorf D, cipotriol and oral retinoids have also been used. Currently our Henz BM, Fuchs PG. Nine-year follow-up of a case of Grzy- own patient is being treated with a topical vitamin D analogue bowski type multiple keratoacanthomas and failure to demon- and remains under regular review. strate human papillomavirus. Br J Dermatol 2002; 147: 793–6). The occurrence of multiple spontaneous giant keratoacan- thomas has been rarely reported and presents a therapeutic challenge.

P-13 A rash and hearing loss V. Madan and R.D. Ead P-12 Salford Royal Hospitals NHS Trust, Lancs, U.K. Keratosis lichenoides chronica presenting in a A 29-year-old woman was referred with a pruritic, raised red paediatric patient rash since birth. She had also developed recurrent fevers, N. Usmani and S.M. Clark intermittent knee joint pains and swellings. In 1994, she had Leeds General Infirmary, West Yorkshire, U.K. a transient episode of left-sided weakness followed by a right- Keratosis lichenoides chronica (KLC) is a rare disorder of ker- sided ischaemic cerebrovascular accident in 2005. She had atinization of unknown aetiology, and is considered by some problems with deteriorating hearing since early childhood. to be a variant of . Infrequent associations with She has been under the care of haematologists for myelodys- hepatitis, tuberculosis, glomerulonephritis and lymphoprolifer- plasia and her mother and two maternal aunts have childhood ative disorders have been reported largely in the adult popula- onset progressive hearing loss. Examination showed wide- tion (Redondo P, Solano T. Keratosis lichenoides chronica in spread erythematous urticated papules and plaques. Systemic childhood. Clin Exp Dermatol 2002; 27: 283–5). We report a examination was normal except for bilateral sensineural hear- paediatric patient with clinical and histological features of this ing loss. Investigations showed a raised ESR (102 mm in the rare condition. A 14-month-old baby girl presented to our first h), CRP (40Æ3 mg L–1), serum IgA 6 g L–1 (0Æ6–4Æ8), IgE clinic with a 2-month history of facial hyperpigmentation. 31Æ5 g L–1 (5Æ4–18Æ2), normal serum IgM, renal function tests There was no preceding history of inflammation or pruritus and low haemoglobin (9Æ3 g dL–1) and lymphocyte count and the rash had progressively worsened despite regular emol- (1Æ3 · 109 L–1). Gene mutation tests failed to show any muta- lients. Her parents were originally from the Cameroon tions. She was treated with anakinra (100 mg daily) subcuta- although the patient herself had been born in the U.K. On neous injection and her skin lesions cleared after two doses. examination the patient had skin type 5 pigmentation with a Muckle–Wells syndrome (MWS; MIM no. 191900) is a domi- reticulated annular, pigmented eruption on both cheeks nantly inherited autoinflammatory disease characterized by

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 30 Bristol Cup Posters rashes, fever, arthralgia, progressive sensorineural deafness and has been described in some cases. To date there are 12 the frequent development of systemic AA amyloidosis. MWS reported cases in the literature of this rare but debilitating is associated with heterozygous mutations in a gene on chro- condition. It was first documented in 1996 in two sisters but mosome 1q44 named NALP3 that encodes cryopyrin which in this is the only reported case with affected family members turn leads to defective apoptosis of neutrophils, leading to (English JC III, McCollough ML. Transient reactive papulotrans- their persistence and inappropriate activation. Interleukin (IL)- lucent acrokeratoderma. J Am Acad Dermatol 1996; 34: 686–7). 1b has a pivotal role in the pathogenesis of this disorder. Since then a further 10 cases have been reported. All cases are MWS belongs to the group of periodic fever syndromes female between 9 and 33 years old, with classical clinical and including familial Mediterranean fever, hyperimmunoglobuli- histological features. In the majority only the palms were af- naemia D syndrome, tumour-necrosis-factor-receptor-associ- fected. We present a case with plantar as well as palmar ated periodic syndrome, familial cold urticaria and neonatal- involvement, in whom there is no resolution when the hands onset multisystem inflammatory disease or chronic infantile are dry. Additionally she has failed to respond to aluminium neurological cutaneous and articular syndrome. Anakinra, a chloride hexahydrate. recombinant nonglycosylated homologue of human IL-1Ra that competitively inhibits binding of IL-1a and IL-1b to the IL-1 receptor type 1, is expressed in a wide variety of tissues and organs. Several manifestations of MWS respond to ana- kinra. Myelodysplasia as a feature of MWS has not been previ- P-15 ously reported. Erythropoietic protoporphyria: delayed presentation and a new FECH mutation L. Smith and N.H. Cox Cumberland Infirmary, Carlisle, U.K. There are a small number of reports of the late presentation of P-14 erythropoietic protoporphyria (EPP), some describing true late Aquagenic palmoplantar keratoderma: a onset of symptoms, and others reporting late presentation of case report and literature review (presumably) mild disease. We present a further such case S. Desai and S. Halpern with a previously undescribed mutation. A 26-year-old Medway Maritime Hospital, Kent, U.K. woman presented with ‘sun allergy’ since the age of 2 years. We present a case of aquagenic palmoplantar keratoderma, the She described burning, itching or tingling of the skin within thirteenth case to be reported in the literature, and present a minutes of exposure to sun, relieved by the application of a summary of the cases reported to date. Our patient, a 23- cold wet flannel. Occasionally she had had redness or swelling year-old Caucasian female presented with a 4-year history of of affected areas, and blisters on one occasion, but usually burning oedematous palms with keratoderma following brief there were no physical signs. Her skin was clinically normal. immersion in water. When dry her palms always remain No family members (parents and female sibling) were mildly keratodermic and have been progressively deteriorating. affected. A diagnosis of EPP was suspected. Routine blood tests She has subsequently also developed plantar involvement. Her were normal; erythrocyte porphyrin level was 22Æ7 lmol L)1 condition was originally diagnosed as aquagenic palmoplantar (normal 0Æ4–17Æ7), and emission spectroscopy of plasma keratoderma in New York in 2003 at the age of 18. Biopsies showed a peak at 628 nm, consistent with EPP. DNA sequenc- taken at the time were found to be consistent with the diag- ing of the FECH gene (Cardiff Porphyria Service) demonstrated nosis. She was subsequently prescribed topical aluminium a mutation c.490C>T, R164W in exon 5. Analysis of the chloride hexahydrate. However her lesions have remained IVS3-48 polymorphic site in intron 3 showed heterozygosity refractory to this treatment. The patient now resides in the (T/C) at this site. R164W is a previously unidentified muta- Middle East where she feels the humid environment has tion that affects a conserved arginine and when in trans to the resulted in further deterioration of her condition. Aquagenic low expression variant IVS3-48 would be expected to cause palmoplantar keratoderma is an acquired, transient, reactive overt EPP. Her sister also had genetic testing with negative condition typically seen in young women. It is characterized results. Treatment with betacarotene 30 mg thrice daily, from by painful oedema of the palms with or without involvement March to October each year, plus sun protection, has given of the soles, with visibly prominent eccrine ducts after expos- relief of symptoms. Reports of such presentations are of ure to water. In some cases there is complete and spontaneous potential interest; they generate the question of whether there amelioration on hand drying, with normal appearing palms. may be genotype–phenotype correlation in severity of symp- The so called ‘hand in bucket’ sign is thought to be pathog- toms (and therefore age at presentation), and also whether nomic. Histology typically reveals a thickened, compact ortho- such presentations may be more common than currently rec- keratotic cornified layer within which there are dilated ognized. acrosyringia. There is dilatation of the eccrine ostia. And the remaining epidermis and dermis are usually unremarkable. Improvement with topical aluminium chloride hexahydrate

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P-16 acute neutrophilic dermatosis. Further investigation is war- Abstract withdrawn ranted to ascertain the pathogenesis of this condition and its natural history.

P-17 P-18 Mid-dermal elastolysis presenting as a persistent Hay–Wells and Rapp–Hodgkin ectodermal dysplasia reticulate erythema syndromes are clinically and genetically indistinct D. Herath, V. Majmudar, S.M. Khorshid and S.E. Clements, T. Hamada, J. Mellerio and M.D. Catterall J.A. McGrath Basildon University Hospital, Essex, U.K. St John’s Institute of Dermatology, London, U.K. A 74-year-old retired chauffeur presented 2 years ago with a Hay–Wells syndrome (HWS, also known as ankyloblepharon- 3-year history of a skin eruption affecting the upper chest, ectodermal dysplasia-clefting, AEC, syndrome; MIM 106260) back and upper arms. It was asymptomatic and there was no and Rapp–Hodgkin syndrome (RHS; MIM 129400) are rare previous history of any skin conditions or autoimmune dis- autosomal dominant disorders characterized by abnormal eases. Over the subsequent 2 years he developed ischaemic development of skin, teeth, hair and nails as well as orofacial heart disease and polycythaemia rubra vera but the rash clefting. Clinical distinction between the two syndromes may remained asymptomatic. On examination he had a rather be subtle; the main differences are ankyloblepharon and scalp widespread, erythematous, annular and arcuate, patchy erup- erosions in HWS and a characteristic facies (narrow nose, tion with a reticulate pattern over the trunk and upper microstomia, midfacial hypoplasia) in RHS. In 2001, muta- limbs. The head and neck were spared. Clinical differential tions in the TP63 (was p63) transcription factor were first diagnoses included a reticular erythematous mucinosis, an reported in HWS and mutations in this gene were also first unusual presentation of sarcoidosis and widespread granu- identified in RHS in 2003. Collectively, thus far 21 different loma annulare. An initial skin biopsy revealed a mild upper TP63 mutations in HWS (n = 13) and RHS (n = 8) have been dermal chronic inflammatory cell infiltrate with no specific documented. Most comprise heterozygous missense mutations features. On clinicopathological correlation and further exam- in the sterile-alpha-motif (SAM) domain located in the tail of ination of the specimen with elastic Van Gieson stain, frag- TP63a-isoforms, a region that is important in p63 protein– mented and thickened elastic fibres were identified in the protein interactions. To extend genotype–phenotype dermis associated with complete loss of elastic tissue in the correlation, we assessed the clinical features and molecular mid-dermis. A diagnosis of mid-dermal elastolysis was made. pathology in three further families (four individuals) with The autoantibody screen was negative. Routine blood tests HWS or RHS. By direct sequencing of genomic DNA, we were normal until recently when platelets have been persis- identified novel missense mutations in the TP63 SAM domain tently elevated due to polycythaemia rubra vera. Mid-dermal in each family: p.G505T, p.L545P and p.G561D. Clinically, elastolysis is a rare entity defined by the selective loss of Proband 1 (p.G505T) had bilateral cleft palate, scalp erosions elastic tissue in the mid-dermis (Patroi I, Annessi G, Girolo- resulting in scarring alopecia, sparse body hair and hypodon- moni G. Mid dermal elastolysis: a clinical, histologic and tia. She was diagnosed as RHS as although she had severe scalp immunohistochemical study of 11 patients. J Am Acad Dermatol erosions, ankyloblepharon was absent. Proband 2 (p.L545P) 2003; 48: 846–51). First described by Shelley and Wood in was diagnosed clinically with HWS on the basis of cleft palate, 1977, it usually presents with well-circumscribed fine wrin- sparse hair and severe scalp and trunk erosions as an infant, kling, perifollicular papular protrusions, or both. To our resulting in subsequent scarring and alopecia. In family 3 knowledge there are only two other reported cases present- (p.G561D), the Proband had bilateral cleft lip and palate, ing with persistent reticulate erythema (Bannister M, Rubel sparse hair, dystrophic nails and scalp erosions supporting a D, Kossard S. Mid dermal elastophagocytosis presenting as diagnosis of HWS. His daughter, also diagnosed clinically with persistent reticulate erythema. Australas J Dermatol 2001; 42: HWS, had a similar phenotype with the addition of unilateral 50–4. Abstract). The aetiology of this condition is as yet ankyloblepharon. The clinical and molecular data in these uncertain but there is evidence to suggest that intense ultra- three families demonstrate the large overlap between HWS violet exposure stimulates the synthesis of elastase and and RHS. Indeed, there is often intrafamilial and interfamilial cathepsin G. It has also been shown that ultraviolet radiation variability for the same TP63 mutations, indicating that other induces matrix metalloproteinase-9 that in turn could be as yet undetermined modifying genes or possible intrauterine involved in the degradation of elastic fibres. MDE has been environmental factors may influence the clinical ectodermal reported in association with autoimmune disorders such as dysplasia syndrome that results. Overall, there seems to be lit- rheumatoid arthritis, Hashimoto’s thyroiditis as well as in tle clinical or molecular justification for maintaining HWS and the presence of positive autoantibodies. There is also evi- RHS as distinct syndromes. An alternative approach to classifi- dence of MDE occurring in response to inflammation such as cation might be to dispense with both terms and to substitute

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 32 Bristol Cup Posters a generic ‘orofacial clefting-ectodermal dysplasia syndrome’ p.G2656V, p.G2375S). Some genotype overlap was noted. We label instead. In conclusion, our study provides further evi- found homozygosity for p.G2369R in a case of recessive tran- dence that HWS and RHS constitute a single genetic entity that sient bullous dermolysis of the newborn, while this substitu- has variable phenotypic expression. Our findings extend the tion has also been observed as a dominant mutation in DEB spectrum of the TP63 SAM domain mutations underlying AEC pruriginosa. We also discovered heterozygosity for p.G2213R and RHS ectodermal dysplasia disorders and demonstrate the in dominant DEB pruriginosa but compound heterozygosity variable expressivity seen in affected individuals. Furthermore, for p.G2213R/c.491delG in generalized recessive DEB. In add- our genotype–phenotype correlation for these individuals sup- ition, heterozygosity for p.G1483D results in no phenotype, ports the consensus that both syndromes represent variable transient bullous dermolysis of the newborn or mild localized clinical manifestations of a common genetic entity. DEB, while homozygosity results in mild recessive DEB. Our study highlights the practical difficulty in precisely separating dominant and recessive glycine substitution mutations in type VII collagen. Nevertheless, the addition of 31 new mutations to the overall COL7A1 mutation database provides a useful resource for improving diagnostic accuracy and genetic coun- P-19 Distinguishing between dominant and recessive selling in this genodermatosis. glycine substitution mutations in the type VII collagen gene (COL7A1 ) in dystrophic epidermolysis bullosa N. Almaani, L. Liu,* P.D.C. Dopping-Hepenstal* and J.A. McGrath P-20 Genetic Skin Disease Group, St John’s Institute of Dermatology, The Guy’s, Aquagenic palmar wrinkling: diagnosis of atypical King’s College and St Thomas’ School of Medicine, London, U.K. and cystic fibrosis in an adult following recognition of *The Robin Eady National Diagnostic Epidermolysis Bullosa Laboratory, this unusual dermatological sign The Guy’s and St Thomas’ NHS Foundation Trust, London, U.K. L.C. Stewart, S. Leech and S.J. Bourke All subtypes of the inherited blistering skin disease, dystrophic Royal Victoria Infirmary, Newcastle upon Tyne, U.K. epidermolysis bullosa (DEB), result from mutations in the type Aquagenic palmar wrinkling (APW) is an uncommon acquired VII collagen gene, COL7A1. Type VII collagen is the major condition, characterized by transient oedematous wrinkling of component of anchoring fibrils at the dermal–epidermal junc- the palm after brief immersion in water. The reaction resolves tion and in DEB the anchoring fibrils show variable numerical within hours and can be associated with discomfort, burning, and/or structural abnormalities, changes that lead to blistering tingling and pruritus. We report a case of APW associated beneath the lamina densa. In the dominant forms of DEB, the with cystic fibrosis (CF) in a 32-year-old female who was not pathogenic mutations typically involve heterozygous (domi- previously known to have CF. She presented to dermatology nant-negative) glycine substitutions within the type VII colla- with a 2-year history of palmar wrinkling upon contact with gen triple helix. In contrast, the molecular pathology of water or her own sweat. Her past medical history included recessive DEB usually comprises nonsense, frameshift or splice one episode of hepatorenal syndrome (cause unknown) asso- site mutations on both COL7A1 alleles. However, some cases ciated with pneumonia, peritonitis and oesophageal varices. of recessive DEB also result from glycine substitutions which She also complained of chronic fatigue and hypothyroidism. may be silent when heterozygous but pathogenic when inher- Clinically she was noted to have white oedematous plaques on ited on both alleles or in trans with another loss-of-function the palms of both hands upon minimal contact with water mutation in COL7A1. As a result, distinguishing between some (< 1 min) and she was diagnosed with APW. Previous reports dominant and recessive glycine substitutions can be very diffi- suggest an association between APW and CF and in view of cult and this can have important implications for making accu- her past medical history she was referred to the adult CF cen- rate diagnoses and for giving appropriate genetic counselling. tre for diagnostic tests and genetic analysis. Her sweat test was To date, about 50 different dominant and about 50 different positive at 70 mmol L–1 and faecal elastase was low at 17 recessive glycine substitution mutations have been reported in lg g–1 indicating pancreatic malabsorption. DNA analysis sub- type VII collagen. Over the last 4 years, we have sequenced sequently revealed two mutations G551D and R1070W con- the COL7A1 gene in > 200 individuals with different forms of firming the diagnosis of atypical CF with an unusual DEB and have identified 31 new glycine substitution muta- phenotype (lack of significant lung disease). She was started tions resulting in either dominant DEB (n = 20; p.G1483D, on appropriate management for CF. The association of APW p.G1649R, p.G1770D, p.G1773D, p.G1776E, p.G1860R, and CF was first described in 1974 and affects predominantly p.G1913R, p.G2009A, p.G2028E, p.G2046D, p.G2067A, females. Worldwide, there have been 31 known reported cases p.G2159E, p.G2213R, p.G2233E, p.G2239V, p.G2260E, of APW (including this case), 18 associated with CF. This case p.G2290A, p.G2360R, p.G2680D, p.G2772R) or recessive and two reported by Katz et al. are the only three cases of DEB (n = 11; p.G1287S, p.G1290E, p.G1338R, p.G1483D, APW/CF with documented gene mutations, nF508 homozy- p.G1851E, p.G1940D, p.G2213R, p.G2272A, p.G2369S, gous CFTR mutation in the two cases reported by Katz et al.

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(Katz KA, Van AC, Turner ML. Aquagenic wrinkling of the X-ray. The ASO titre was markedly elevated on several occa- palms in patients with cystic fibrosis. Arch Dermatol 2005; 141: sions (287–528 kIU L–1). Daughter 2, aged 24, was less 621–3). Our case is the first to report a heterozygous gene severely affected. She described twice yearly episodes of mutation. The pathogenesis and aetiology of APW is lesions, primarily affecting the dorsum of her hands, with unknown. Patients with CF have abnormally increased epider- flares during pregnancy. She had marked arthralgia, particularly mal salt concentrations which may play a role in the develop- affecting her knees. FBC, inflammatory markers, rheumatoid ment of APW. Other possible theories include abnormal factor, autoantibody screen and Mantoux test were negative. electrolyte fluxes, defective barrier function of stratum corneum, She has been referred to for further investigation occlusion of eccrine ostia, weakness of eccrine duct wall and of her joint pain. Screening tests for causes of EN were nega- influx of water across an osmotic gradient into eccrine ducts. tive in this family. A family with four affected female members We present this patient to highlight the importance of consid- was reported, in which all episodes were associated with phar- ering the diagnosis of CF, a disease of significant mortality yngitis. Three male siblings in this family were unaffected and morbidity, in patients with APW. Our patient was diag- (Elkayam O, Caspi D, Segal R et al. Familial erythema nodosum. nosed aged 32 with atypical asymptomatic CF which would Arthritis Rheum 1991; 34: 1177–9). Familial idiopathic EN has not have been diagnosed without the recognition of this un- rarely been reported, all cases to date being female. We report usual dermatological sign. CF should be considered in all the third family (mother and two daughters) with idiopathic patients with APW. EN. In this family arthralgia is a prominent feature.

P-21 P-22 Familial idiopathic erythema nodosum The objective structured clinical examination: an B. Moran, M. McAleer and F.C. Powell opportunity for formative as well as summative Mater Misericordiae University Hospital, Dublin, Ireland assessment Familial idiopathic erythema nodosum (EN) is rare, and has J. Sansom, L. Dow,* S. Wensley* and A. Blythe only been reported in female patients. Arthralgia is a common Bristol Dermatology Centre, Bristol, U.K.; *Frenchay Hospital, Bristol, U.K. association. We present the case of a family of six, in which and University of Bristol, Bristol, U.K. all three female members developed recurrent EN. Patient 1, a It is possible for individuals to pass exams without knowing woman aged 50, reported a 37-year history of monthly epi- what has been done well and what could be improved upon. sodes of tender, erythematous lesions affecting her limbs. The objective structured clinical examination (OSCE) is widely These were relieved by multiple short courses of oral steroids. used in U.K. medical schools to assess attitudes, clinical skills Aspirin was ineffective. Episodes were associated with fatigue, and knowledge. In National Student Surveys our undergradu- myalgia and arthralgia. A biopsy was consistent with EN. ates rated the feedback that they received during their training Computed tomography of the thorax, abdomen and pelvis, as poor. We report the development and evaluation of using chest X-ray, C-reactive protein, a1-antitrypsin, extractable the OSCE to provide both formative and summative assess- nuclear antigens (ENA), antinuclear antibodies (ANA), ment to 4th year undergraduate students. Our 10-week Unit rheumatoid factor, joint X-rays, full blood count (FBC), urea combines teaching and learning in Primary Care, Dermatology and electrolytes, liver function, amylase and angiotensin-con- and Care of the Elderly. As part of their summative assess- verting enzyme (ACE) were normal. A Mantoux test was nega- ment, students sit a 10-station OSCE, with additional rest sta- tive. The erythrocyte sedimentation rate was 33 mm in the tions. We have used this setting as an opportunity for station first hour. She described frequent ‘sore throats’. Antistreptoly- examiners to provide formative feedback to students immedi- sin O (ASO) titre had been mildly elevated on several occa- ately following their exam. Students complete the OSCE dur- sions (126–135 kIU L–1), but prolonged antibiotic cover ing which each station examiner makes notes concerning each failed to prevent lesions developing. Throat swabs were con- student’s performance. At the end of the OSCE students circu- sistently negative and a DEXA scan revealed osteopenia. Her late again around the OSCE stations to receive formative feed- two daughters (aged 26 and 24 years) had recurrent EN, start- back from their examiner at each station, although the ing about the age of 13 years. Daughter 1, aged 26, also summative mark is not disclosed. We initially allowed 1 min- described monthly flares, with lesions affecting her limbs. Epi- ute for feedback at each station and, following student and sodes were associated with arthralgia. Biopsy showed pannicu- examiner feedback, have now increased this to 2 minutes. Stu- litis, consistent with EN. She also had multiple courses of oral dents were asked to rate the usefulness of the formative assess- steroids, and was diagnosed with osteopenia. Her symptoms ment on a Likert scale. Of the 99, 94, 117 and 109 students improved with aspirin. The flares were more frequent when sitting consecutive exams, response rates (%) were 89, 87, 72 she took the oral contraceptive pill. Baseline blood tests were and 74. The results following the 2 minute feedback are as normal, as were ENA, ANA, rheumatoid factor, ACE and chest follows (all results given as percentages).

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 34 Bristol Cup Posters

policies under the aegis of student welfare coordinators. Not Very None of Although the risk of sunburn in the U.K. is significantly less useful Slightly Moderately useful these than that in Australia there are lessons which could be learnt Feb 2006 1 1 25 68 3 which would encourage schools to take a more active role in Jul 2006 0 7 35 57 0 Feb 2007 0 6 23 71 0 protecting young children’s skin as current recommendations Jul 2007 1 5 27 67 0 are clearly not being implemented.

The OSCE is a time-consuming and resource-intensive exam. Examiners need specific, detailed instruction and training so that they can give accurate feedback subsequently. We have demonstrated that using this opportunity to provide timely P-24 Training dermatology medical students in effective and specific formative assessment is perceived as valuable by and nonpatronising communication with disfigured our students and could be used elsewhere in the under- individuals: a combined approach between a graduate programme. university medical school and Changing Faces R. Healy, A.P. Bewley and C. Wheatley* Barts and the London NHS Trust, London, U.K. and *Changing Faces, London, U.K. Disfigurement affects approximately 400 000 people in the P-23 U.K., and is a common reason for referral to dermatology Sun protection in schools units. Causes of disfigurement can be congenital, as a result of B. Grange, E. Veysey, M. Morris and D.L.L. Roberts cutaneous trauma and (often dermatological) disease. The psy- Singleton Hospital, Swansea, U.K. chosocial implications of disfigurement are enormous. The Sun protection during childhood and adolescence is the most Dermatology Department at Barts and the London (Queen critical factor in minimizing the risks of skin cancer. The inci- Mary Medical School, University of London) have, together dence of skin cancer in South Wales is rising, increasing by with the charity Changing Faces (www.changingfaces.org.uk 74% between 1986–8 and 1996–8 (Holme SA, Malinowsky accessed 19 February 2008), set up a training programme K, Roberts DL. Changing trends in non-melanoma skin cancer which aims to educate medical students in the day-to-day in South Wales. Br J Dermatol 2000; 143: 1224–9). It is also implications of living with disfigurement, effective dialogue the highest recorded incidence rate in the U.K. and one of the with a disfigured individual, and (where sought) appropriate highest in Europe. Consequently it is critical to examine the management of disfigurement. A yearly training day, in year sun-protection measures taken in schools and to see how they one of a 5-year medical school curriculum, has been set aside can be improved. Cancer Research UK runs the SunSmart pro- for the previous 8 years. The yearly intake of medical students tection policy encouraging schools to implement sun-protec- has been 125 (1998) increasing to 210 (2007). Training is tion measures. A survey was sent to the local nurseries, provided by dermatologists and senior lecturers (University of primary and secondary schools in order to assess how they London), together with lecturers from Changing Faces (a complied with the SunSmart guidelines. The survey included charity specifically set up to address the psychological issues such factors as: (i) whether a sun protection policy was in affecting individuals with a disfigurement). Practical sessions place, (ii) what types of shade were provided, (iii) changes in are initially interactive (students are invited to discuss personal timetables to avoid heavy sunlight, (iv) advice on clothing responses to a range of disfigurements). An interactive training and sunscreen as well as supervision with regards to applica- session is then given which establishes (through a range of tion of sunscreen. The survey found that in many categories techniques such as role-play, didactic teaching and student- the schools fared poorly with primary and comprehensive centred learning) best practice in effective, nonpatronising schools faring particularly badly: (i) less then half of compre- communication with disfigured individuals. Feedback from hensive and primary schools had a sun-protection policy in the medical students has indicated (year on year) that the place, (ii) schools relied on incidental rather than purpose- learning experience has been invaluable. Changing Faces is a built shading, such as trees and buildings, (iii) many children charity, actively involved in research, with a specific remit to were allowed out at any time during the day, (iv) children challenge the way disfigurement is perceived. We believe we were not reminded to use sunscreen. Australia is a world lea- have developed an innovative and essential training tool with der in sun-protection strategies and as such can be used as a a specific remit to train students, early in their training, in model for improving strategies in the U.K. It has used both effective and nonpatronising communication with disfigured specific and general community programs including mass individuals. media campaigns, changes to legislation and use of nurses to supplement education within schools. In addition, the KidSkin protection program has created SunSmart schools, schools which have successfully implemented detailed sun-protection

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 35

P-25 perception of disfigurement (even within a dermatological Differences in the perception of cutaneous training framework) is personal, self-referenced but also disfigurement between medical students dynamic (www.changingfaces.org.uk accessed 19 February and general practitioners 2008). R. Healy, A.P. Bewley and C. Wheatley* Barts and the London NHS Trust, London, U.K. and *Changing Faces, London, U.K. Over 400 000 individuals in the U.K. live with a disfigure- ment, often with profound psychosocial implications. We P-26 have established training modules for both medical students Eleven years’ experience of simplified surgical and general practitioners (GPs) to facilitate empathic and non- treatment of chondrodermatitis nodularis by patronising communication with disfigured individuals. Over cartilage trimming and sutureless skin closure the previous 5 years we have compared medical students’ per- W. Hussain and R.J.G. Chalmers ception of disfigurement with that of GPs. Over a 5-year per- Dermatology Department, Bolton Royal Infirmary, Bolton, U.K. iod, 785 medical students and 251 GPs, were asked to Chondrodermatitis nodularis (CD) is a common ear condition identify which, from a series of six images of disfigured indi- caused principally by focal pressure on the skin of the pinna viduals, they found to be most disfigured. The images were from the weight of the recumbent head. It presents as a pain- consistently the same throughout the study period, and com- ful nodule or skin ulcer located at the point of maximum prised a male youth with severe acne, a man with HIV and pressure from underlying pinnal cartilage and, although most facial Kaposi’s sarcoma, a baby with a bathing trunk naevus, commonly seen at the apex of the helix, varies in location an Asian man with facial vitiligo, an Afro-Caribbean woman according to the shape of the pinna. Age, skin damage from with extensive neurofibromatosis and a Caucasian man with cold or chronic sun exposure and an inability to sleep other extensive scalp alopecia areata. Results were consistently simi- than on one side are predisposing factors. Various surgical and lar within medical student and GP groups, but there were sig- nonsurgical treatments have been advocated for CD; however nificant differences between medical students and GPs in their excision of cartilage alone as originally described by Lawrence perception of disfigurement. Of the medical students, 51% has been shown to be therapeutically and cosmetically effec- (mean) compared with 8% (mean) of GPs thought that the tive (Hudson-Peacock MJ, Cox NH, Lawrence CM. The long- image of the youth with severe acne most disfiguring, 33% of term results of cartilage removal alone for the treatment of medical students compared with 5% of GPs found the image chondrodermatitis nodularis. Br J Dermatol 1999; 141: 703–5). of an HIV-affected man with Kaposi’s sarcoma most disfigur- Since 1996 we have treated the majority of patients with CD ing, 10% of medical students compared with 33% of GPs using a modified version of the Lawrence technique. Sufficient found the image of a patient with neurofibromatosis most dis- local anaesthetic is injected to lift the skin from underlying figuring, 5% of medical students and 5% of GPs found the cartilage. For the helix, a short horizontal skin incision is image of a patient with bathing trunk naevus most disfiguring, made along the edge of the helical sulcus underneath the af- < 1% of medical students compared with 5% of GPs thought fected area to allow access to the cartilage, which is scored the image of a patient with alopecia areata most disfiguring lengthwise with a scalpel blade. Mosquito forceps are used to and < 1% of medical students and 33% of GPs found the remove the released cartilage fragments until the helical rim image of a patient with vitiligo most disfiguring. Human feels completely smooth on palpation. SteristripsÒ (3M Health- visual perception is highly complex and involves visuospatial care Ltd., Loughborough, U.K.) are used to advance skin from recognition together with a complex range of higher cognitive the obverse of the helix over the helical rim and into the hel- functions. Cognitive perceptive functioning is thought to be ical sulcus to close the wound. Skin nodules are not removed dynamic, and highly influenced by personal, cultural, experi- but resolve spontaneously. The average time taken for the pro- ential and environmental factors (Thompson A, Kent G. cedure is 15 min. Of 33 patients treated over 11 years using Adjusting to disfigurement, processes involved in dealing with this technique, 19 were men (mean age 61 years; range 47– being visibly different. Clin Psychol Rev 2001; 21: 662–82). 83) and 14 were women (mean age 65 years; range 48–92). Also, it is recognized that empathy involves self-reference. We Right (n = 17) and left (n = 15) ears were almost equally rep- found that, over a 5-year period, successive groups of medical resented. One patient required treatment for both ears. The students consistently (mean 51%; range 39–55%) thought the antihelix was involved in seven cases; the remainder involved image of the youth with severe acne the most disfiguring of the helix. All patients were reviewed at 4 months. An excellent the six images they were shown. This compares with a con- response with no discomfort or clinical recurrence of CD was sistently smaller percentage of GPs (mean 8%; range 7–10%) observed in 31 (93%). These 31 patients were all given open shown the same images. Medical students, being closer to the appointments to re-attend should symptoms recur but none age when, for example, acne is likely to be more personally required to be seen again. The Lawrence technique of cartilage relevant, found the greatest disfigurement in an image of an removal offers long-term cure rates in excess of 80%. By using individual with severe acne. GPs found the greatest disfigure- a short incision within the helical sulcus and avoiding the ment in other images. Our findings support the concept that need for sutures, we believe we have improved and simplified

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 36 Bristol Cup Posters the procedure and have confirmed the high success rate of student feedback is to structure a rotation so that students this approach to management of a common and disabling have the opportunity to clerk and present in clinic, backed up condition. by a theoretical component to ensure consistency of teaching and student experience.

P-27 What medical students value from a specialist P-28 dermatology rotation: course evaluation from Evaluation of a dermatology course for medical a U.K. medical school students delivered in four clinical academy settings E. Topham, C. DeGiovanni and C.R. Darley C.B. Archer Brighton and Sussex Medical School, East Sussex, U.K. Bristol Dermatology Centre, Bristol Royal Infirmary (UBHT), Bristol, U.K. Research in medical education has shown that adult learners Thirty-eight medical students were asked to evaluate their value active involvement in their learning and that a variety of dermatology course by completing a detailed questionnaire. teaching techniques is desirable in order to engage learners They received 10 introductory lectures in the university with different learning styles. Clinical experience is an essen- (teaching) hospital. In response to increased student numbers, tial part of medical training. However teaching in clinical set- this was the first year of a ‘clinical academy’ system in which tings can be challenging because of its opportunistic nature, the students received clinical teaching in either the Teaching time restraints and space limitations as well as the possible Hospital, two District General Hospitals or a General Practice reluctance of patients to be involved. The curriculum for the (GPwSI) setting. The objectives were as follows: to evaluate 15-day, year 4 dermatology specialist rotation is based on the the dermatology course by seeking questionnaire responses in BAD undergraduate dermatology curriculum. Timetabled activ- relation to the effectiveness of different teaching formats, ities per student include eight clinics, surgical, phototherapy, including lectures, timetabled outpatient clinics, optional add- patch test and leg ulcer sessions, a video session, student-led itional outpatient clinics, GPwSI clinics, teaching ward rounds, case presentations, intranet-based clinical case scenarios and small group sessions (tutorials) and reflective sessions; to six half-day workshops. Workshops cover history and exami- establish if and how teachers were formally evaluating their nation and common or important presenting complaints. All individual teaching sessions and giving feedback to the stu- students clerk and present new patients from day 1; teaching dents involved in small group presentations; to determine clinic templates are reduced to allow this. Students must com- whether student performance in the dermatology assessment plete a skills and attendance logbook. All 106 fourth year after the course was influenced by where they learnt about medical students were asked to complete an evaluation ques- skin disorders; to find out what time was spent in self-directed tionnaire on the last day of their attachment and 91 (86%) learning, what resources were used, and whether students felt responded. Students were asked general questions about the that the course met its learning objectives and their learning rotation and then asked to score individual teaching episodes needs. The questionnaire was designed to be completed in on a Likert scale. Free text explanation for the scores was also 10–15 min, and was handed out and collected at the end of a requested. The highest scoring (scored 1–5) teaching sessions reflective teaching session, thereby increasing the rate of for usefulness were changing lesion clinics (4Æ8) and general return (37 out of 38). There were a combination of closed clinics (4Æ7), followed by presenting complaint-based work- questions, open-ended questions and scale items. Responses shops (4Æ5) and intranet case scenarios (4Æ0). Surgical sessions were coded for analysis. Each group of students ‘agreed’ scored 3Æ3 and video sessions 3Æ0. Twenty-nine students (score of 4) that lectures, teaching ward rounds and small (32%) said they would consider a career in dermatology, and group sessions were effective ways to learn about dermatol- only two students (2%) said they felt unprepared to deal with ogy. Students ‘strongly agreed’ that hospital outpatient clinics, dermatology patients as a foundation doctor. From both the reflective sessions and small group sessions in one DGH were Likert scores and free text feedback, clinic experience was the effective. The scores for the effectiveness of GPwSI clinics as a most valued aspect of the rotation. The overwhelming reason teaching format were consistent with an overall ‘not sure’ given was that students were actively involved with clerking response. Students ‘agreed’ that the group demonstration for- patients and receiving feedback. Active participation followed mat was more effective than a lecture format in the reflective by feedback is well documented in medical education litera- sessions. Formal evaluation of large group sessions was sought ture to be an effective way of learning. The workshops were much more frequently than for each of the small group teach- also valued highly. These covered the learning outcomes of ing formats. Of 30 students clerking patients in a hospital set- the curriculum and were all presenting complaint-based so ting, 25 received feedback about their performance. Multiple were perceived as relevant. Most workshops were interactive choice questionnaire scores for one DGH clinical academy and had student-led components. Other teaching areas scored group were significantly lower (P < 0Æ05) than in the two less well as they were less interactive (surgery) or perceived as other hospital settings. Students in the three hospital settings less directly relevant (videos). The key message from the commented that the course learning objectives had largely

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 37 been met but students in the GPwSI setting expressed some P-30 concerns over their lack of exposure to dermatology in a hos- Analysis of volatile compounds: a new and rapid pital secondary care setting. This situation has since been cor- method to detect and differentiate dermatophytes rected. K. Naraghi, B. Adriaans,* H. Barr* and N. Magan Cranfield University, Cranfield Health, Bedford, East, U.K. and *Gloucestershire Royal Hospital, Gloucester, South West, U.K. Dermatophytoses are common among skin diseases in both developed and developing countries. Laboratory confirmation of these infections is mostly based on the isolation of derma- P-29 Dermatology education in Ireland tophytes from cultures. Culture, however, is very time-con- A.-M. Tobin and C. Buckley suming and depends on the expertise of laboratory staff. Thus, Waterford Regional Hospital, Waterford, Ireland there is a need for alternative techniques that are quick and Each year approximately 500 junior doctors graduate from Ire- highly specific. All micro-organisms produce organic com- land’s five medical schools. In a survey of dermatology educa- pounds as a result of their normal metabolism. Some are vola- tion all students receive teaching in dermatology as tile at low temperature and may have characteristic odours. undergraduates. This consists of formal lectures and clinical These volatile organic compounds (VOC) can potentially be attachments which may or may not be available to all stu- used as biomarkers quickly to detect and differentiate human dents. We undertook a survey of awareness of dermatological pathogens including dermatophytes (Turner AP, Magan conditions in patients admitted both surgically and medically N. Electronic noses and disease diagnostics. Nature Microbiol Rev to a 400-bed acute hospital by nonconsultant hospital doctors. 2004; 2: 161–6; Sahgal N, Monk B, Wasil M, Magan Patients who had been admitted from the emergency depart- N. Trichophyton species: use of volatile fingerprints for rapid ment were approached and asked would they be willing to identification and discrimination. Br J Dermatol 2006; 155: undergo a full skin examination by a member of the derma- 1209–16.). The objective of this work was to use an elec- tology department. A history of any skin conditions was also tronic nose (E-nose) system to detect and qualitatively analyse recorded for each patient. The patient’s medical records were the volatile fingerprints of dermatophytes in clinical samples then accessed and the admission notes reviewed to ascertain and isolates as a rapid and accurate alternative diagnostic any reference to skin findings. A questionnaire was circulated method to culture. To achieve this objective, control dermato- to all medical staff, consultants, registrars, senior and junior phytes, clinical samples and harvested spores from clinical iso- house officers, to assess the quality and quantity of undergrad- lates were inoculated in culture medium and after 96–120 h, uate training in dermatology. Fifty-seven patients and their the VOC patterns were analysed by an E-nose system. Results, notes were audited, 43 patients had been admitted under the using principal component analysis, showed that the E-nose care of medical teams and 14 patients under the care of the was able to differentiate between positive and negative con- surgeons. There were 34 males and 23 females with an aver- trols, clinical samples and clinical isolates as early as 96 h of age age of 64 years (range 27–99). In 42 patients there was growth. This accounted for 98% of the data in principal com- no reference to skin conditions either in the systems review or ponents 1 and 2 based on the qualitative volatile production the clinical examination in the admission notes. Of these 42 patterns. Results were also confirmed by cluster analysis of the patients, 33 had findings on full skin examination, frequently data using Euclidean distance and Ward’s linkage. This is the these were benign lesions such as warts, seborrhoeic derma- first detailed study of the use of an E-nose system to detect titis and discoid eczema. However two melanomas, a lentigo and differentiate dermatophytes in clinical samples. This tech- maligna, four clinical squamous cell carcinomas and four clin- nique can significantly decrease the amount of time needed to ical basal cell carcinomas were not recorded. Three patients in confirm the diagnosis of dermatophytoses, and therefore will this group were also renal transplant patients; fortunately on enable clinicians to start effective treatment and control pro- examination these patients only had warts. In a survey of grammes earlier. medical staff involved in acute admissions, 70% felt they did not feel competent to describe or manage dermatological con- ditions and would welcome additional education and training in dermatology. The dermatological content of the undergrad- uate medical curriculum in the U.K. has recently been defined; P-31 this may facilitate adapting a curriculum (Clayton R, Perera R, Panton–Valentine leucocidin-producing Burge S. Defining the dermatological content of the under- Staphylococcus aureus causing recurrent graduate medical curriculum: a modified Delphi study. Br J furunculosis in an immunocompetent patient Dermatol 2006; 155: 137–44). It is also possible that some T. Joseph, S. Jain and C.J.M. Stephens teaching might be undertaken at postgraduate level. Poole Hospital NHS Trust, Poole, U.K. Panton–Valentine leucocidin (PVL) is a cytotoxin produced by some strains of Staphylococcus aureus that causes leucocyte destruction and tissue necrosis, producing a new pattern of

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 38 Bristol Cup Posters disease in the U.K. and worldwide. It is produced by fewer lamisil after mycology confirmation. Both were treated suc- than 2% of S. aureus strains and can be carried by both methi- cessfully. A review of all zoophilic dermatophyte infections in cillin-resistant (MRSA) and methicillin-sensitive Staphylococcus the West and parts of North Scotland in the period 1996– aureus (MSSA) (Lina G, Piemont Y, Godail-Gamot F et al. 2006 was performed. One hundred and four culture-positive Involvement of Panton-Valentine leukocidin producing Staphy- zoophilic dermatophyte specimens were reported. lococcus aureus in primary skin infections and pneumonia. Clin Infect Dis 1999; 29: 1128–32). We report a patient with recur- Organism Source Incidence rent boils on her leg and axillae caused by PVL-producing Microsporum canis Cat and dog 53 (50Æ9%) MSSA. A 24-year-old lady presented with a history of recur- Trichophyton mentagrophytes var. mentogrophytes Rodent 39 (37Æ5%) rent boils on her legs since December 2006, with the onset in Trichophyton verrucosum Cow 10 (9Æ6%) Borneo while backpacking abroad. More recently, boils had Trichophyton erinacei Hedgehog 2 (1Æ9%) developed in both axillae. Medically she was fit with no his- tory of diabetes. A swab taken from the axillae grew PVL+ The age range of the patients was 1–84 years (median age of MSSA and she was treated with a 1-week course of flucloxacil- 25Æ1). There were 69 female patients and 35 males. Direct lin by her GP. She was referred to the Dermatology Depart- microscopy was positive in 61 specimens (59%). Microsporum ment as she continued to develop active boils. A nasal swab canis remains the commonest zoophilic organism followed by was negative. We followed advice from the Health Protection T. mentagrophytes var. mentagrophytes with the majority of cases Unit regarding treatment. Flucloxacillin is the drug of choice found in young people. This trend of zoophilic dermatophyte for PVL+ MSSA, but this organism is also sensitive to tetracy- infections has remained unchanged over the last three decades. cline, hence a 1-month course was recommended, in addition An explanation for this is that both organisms are prevalent in to chlorhexidine wash and mupirocin ointment for the nose. companion animals. In cases of tinea capitis however the inci- Her partner was also treated with chlorhexidine and mupiro- dence of T. tonsurans and T. violaceum has been rising whereas M. cin ointment. She is currently symptom free. PVL is mainly canis as a causative organism, has been declining (Borman AM, associated with cellulitis and skin infections such as abscesses, Campbell CK, Fraser M, Johnson EM. Analysis of the dermato- boils and carbuncles and may mimic hidradenitis suppurativa phyte species isolated in the British Isles between 1980 and (Durupt F, Mayor L, Bes M et al. Prevalence of Staphylococcus aur- 2005 and review of worldwide dermatophyte trends over the eus toxins and nasal carriage in furuncles and impetigo. Br J last three decades. Med Mycol 2007; 45: 131–41). Dermatol 2007; 157: 1161–7). Rarely it may produce severe infections such as septic arthritis, bacteraemia and highly lethal necrotizing pneumonia in young immunocompetent patients. The incidence of PVL-producing Staphylococcus is low at present but it is important for healthcare professionals to be aware of the infection it can cause and the preventative mea- P-33 sures which need to be taken. Ecthyma gangrenosum: a valuable aid to the clinical diagnosis of Pseudomonas aeruginosa bacteraemia A.B. Alexandroff, A. Iqbal* and W.J. Loo* Leicester Royal Infirmary, Leicester, U.K. and *University Hospital of North Durham, Durham, U.K. P-32 Ecthyma gangrenosum is a rare condition with characteristic Review of all zoophilic dermatophyte infections cutaneous findings in patients with life-threatening Pseudomonas in the West and North of Scotland in the period aeruginosa bacteraemia. It usually affects immunocompromised 1996–2006 patients and requires prompt and aggressive treatment. A 55- F. Al-Niaimi, C. Jury, A.D. Burden, M. Mealyea and year-old woman with chronic lymphocytic leukaemia was G. Shankland admitted with fever (38Æ4 oC) and hot painful urticated pla- Alan Lyell Dermatology Department, Glasgow, U.K. ques with central haemorrhagic blisters. The general medical Dermatophyte infections occur commonly and can be caused examination and screen for sepsis were normal except for by zoophilic, geophilic and anthrophilic dermatophytes. We neutropenia (0Æ2 · 109 L–1) and a CRP of 360 mg L–1. P. aeru- present two cases of dermatophyte infections caused by pet ginosa sepsis with ecthyma gangrenosum was diagnosed and rodents (one of which was caused by a chinchilla). Skin scrap- treated aggressively with gentamicin and piperacillin with ings obtained from both patients showed Trichophyton mentagro- tazobactam, which led to the rapid resolution of the systemic phytes var. mentagrophytes to be the causative organism. Both features and cutaneous lesions. A skin biopsy showed nonspe- patients presented with well-demarcated pruritic areas of ery- cific necrosis but the diagnosis was confirmed by the isolation thema and scaling. The first patient (aged 11) presented with of P. aeruginosa from the skin biopsy and blood cultures. Immu- a patch of tinea on his neck whilst the other patient (aged 40) nocompromised patients are particularly susceptible to P. aeru- had a patch of tinea on her forearm. Both patients were trea- ginosa sepsis but it can also occur in previously well children ted with topical lamisil, although the first patient received oral (Goolamali S, Fogo A, Shaikh H et al. Echthyma gangrenosum:

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 39 an important sign of potentially fatal Pseudomonas sepsis and a had received antileprosy treatment before presentation. All the rare case occurring in a previously well child. Br J Dermatol patients were treated successfully with WHO multidrug ther- 2007; 157 (Suppl. 1): 127; Freiman A, Nguyen K, Muhn CY apy (MDT). As the bacillary load is very high in these patients, et al. Erythematous groin plaque in a patient with multiple they can form a potential reservoir of the infection in the myeloma. CMAJ 2004; 171: 32). Ecthyma gangrenosum usu- community especially in the postleprosy elimination era. Con- ally begins as a painless erythematous macule, which develops trary to the earlier belief in the dapsone era, the majority of into a haemorrhagic bulla and rapidly evolves into gangrenous the patients manifest disease de novo without any history of anti- ulcerations with black eschars surrounded by an erythematous leprosy therapy or suggestion of drug resistance. A high index halo. The histology typically shows acute inflammation of the of suspicion is required for the diagnosis of such cases at the media and adventia of the venules but sparing the intima. In present time when leprosy management is being integrated view of the grave prognosis, early recognition and aggressive with general health services and the special expertise for the treatment of this condition are critical. Although ecthyma gan- diagnosis and management of leprosy is becoming extinct. grenosum occurs in a minority of patients with P. aeruginosa bacteraemia, the characteristic appearance of urticated plaques with central blistering and necrotic ulceration can serve as a valuable aid to this important diagnosis.

P-35 Sleeping with the enemy D. Sandhu, K. Batta, F. Tatnall and C. Boase* P-34 Watford General Hospital, West Hertfordshire Hospitals NHS Trust, U.K. Histoid Hansen’s disease in the postleprosy and *The Pest Management Consultancy, Haverhill, Suffolk, U.K. elimination era: a report of six cases and The relationship between bed bugs and humans has been doc- their epidemiological significance umented as far back as the time of the Egyptians and in the S. Dogra, I. Kaur, D. De and U.N. Saikia* 1930s most houses in the U.K. had infestations. From the Departments of Dermatology and *Pathology, Postgraduate Institute of 1950s there was a gradual reduction in their number in devel- Medical Education and Research, Chandigarh, India oped countries and they were rarely seen. However since the Histoid leprosy is an uncommon multibacillary form of dis- mid 1990s the incidence of bed bugs has been steadily rising. ease with unique clinical, bacteriological, immunological and A prospective cohort study revealed an annual 25% increase in histopathological features. Patients with histoid Hansen’s dis- bed bug reports to London borough councils from 2000– ease generally remain undetected for a long time and their 2006 (personal communication C. Boase, The Pest Manage- lesions are often confused with other dermatoses. Its occurrence ment Consultancy, Haverhill, Suffolk). We have seen three in the postleprosy elimination era raises serious epidemi- patients presenting with bed bug bites in a 3-month period. ological concern for spread of infection in the community. In These patients all had an itchy rash mainly affecting the limbs, this prospective study from January 2006 to December 2007, comprising urticated papules and plaques. All three patients six new cases of histoid Hansen’s disease were diagnosed at brought in the causative insects to the clinic and they were our centre, based on criteria suggested by Sehgal and Srivast- identified as Cimex species bed bugs by our microbiology ava (Sehgal VN, Srivastava G. Status of histoid leprosy – a clin- department. Two patients had recently purchased second-hand ical, bacteriological, histopathological and immunological furniture for their homes. Additional family members were appraisal. J Dermatol 1987; 14: 38–42) and Wade (Wade HW. affected in two of the index cases. All patients were treated The histoid variety of lepromatous leprosy. Int J Lepr 1963; 31: symptomatically with antihistamines and topical steroids and 129–42). Data regarding demographic details, clinical features, advised to contact the council environmental health depart- treatment, complications and the course following treatment ment to arrange eradication of the infestation. Of the three were noted. Histoid leprosy comprised 2% of the total regis- patients, one paid the council £59 for chemical spray treat- tered leprosy cases during this period at our centre. These ment with a good result; another refused to pay the council were five male and one female patients of mean age 37 years cost of £160 and is self-treating with chemical and nonchemi- while the mean disease duration was 3Æ5 years. Of these, five cal approaches but has persistent infestation; the third patient patients had a primary diagnosis of lepromatous leprosy and has moved to a new location with no further problems. The de novo histoid lesions appeared in one patient. Nodules or sub- temperate bed bug, Cimex lectularius, is a blood-sucking ectopar- cutaneous nodules were the commonest type of lesion seen in asite. Bites are accompanied by the injection of vasodilatory five of the six patients. The average baseline bacillary index and anticoagulant agents into the host. The host reaction can (BI) was 4Æ8, ranging from 3 to 6. The most common ana- range from no response to purpuric macules, urticarial pap- tomical area of involvement was the thighs and buttocks ules, bullae and rarely an anaphylactoid reaction. Despite their (65%). Mucosal involvement was not observed in any patient. blood-feeding behaviour, they are not thought to act as a Nerve function impairment (sensory and/or motor) was most vector for blood-borne pathogens. However, in chronically commonly noted to involve the ulnar nerve. Only one patient heavily infested parts of the world, they are thought to con-

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 40 Bristol Cup Posters tribute to the development of iron-deficiency anaemia. In add- required introduction of either terbinafine or griseofulvin. ition there can be significant psychological consequences. Sev- Two patients admitted to poor compliance and one individual eral factors are thought to aid the successful resurgence of bed relapsed 4 months after the discontinuation of treatment. Of bugs, in particular, bed bug resistance to insecticides. Other the 31 patients with confirmed Trichophyton species, six were factors that may contribute are growth in international travel, lost to follow-up or were noncompliant, 14 of the remaining growth of second-hand furniture sales, more central heating 25 were cured clinically after less than 6 weeks of treatment, and double-glazing and a move away from broad-spectrum and five after 6–12 weeks. Of the three patients with con- insecticides. Infestations are currently not notifiable, and treat- firmed M. canis, one patient had a partial response after 6 ment options, which may be chemical, e.g. sprays, and/or weeks of oral itraconazole and two patients were unresponsive nonchemical, e.g. heat or steam treatment, can be carried out after up to 11 weeks of treatment and required the initiation by the resident or a professional pest control organisation. of other antifungal treatments. Fungal cultures, which were Given the increased difficulty in eradicating infestations repeated in 12 patients whose initial culture was positive, attempts are being made by pest control experts to identify were clear in 11. Itraconazole was well tolerated in all cases and agree best practice. with no recorded side-effects. In summary, clinical cures were obtained in 83% of our total cohort (for whom follow-up was available) after up to 12 weeks of treatment with itraco- nazole 3–5 mg kg–1 daily. Cure rates of 76% were obtained amongst those with confirmed Trichophyton species, but cure P-36 rates among those with M. canis infection were much lower. Experience with oral itraconazole in the manage- ment of childhood tinea capitis over a 2-year period C. Cusack, A.A. Syed, R. Watson and A.D. Irvine Our Lady’s Children’s Hospital, Crumlin, Dublin, Ireland Tinea capitis is a common paediatric infection for which gris- eofulvin is the only licensed medication. However, newer P-37 treatments, while not licensed for tinea capitis, may have simi- Pseudo-Pseudomonas dermatitis lar efficacy to griseofulvin and are readily available (Gonzalez S. George, N. Desai,* J. Shepherd and C.C. Harland U, Seaton T, Bergus G et al. Systemic antifungal therapy for Epsom and St Helier NHS Trust, Carshalton, Surrey, U.K. and *St George’s tinea capitis in children. Cochrane Database System Review Healthcare NHS Trust, Tooting, London, U.K. 2007; CD 004685). We conducted a retrospective study Pseudomonas dermatitis is associated with exposure to contami- which investigated the efficacy and safety of oral itraconazole nated water in whirlpools and swimming pools. The nomen- in the management of tinea capitis; 86 children who attended clature is confusing as it represents a rather than a our department from January 2005 to June 2007 were true dermatitis. We describe a new variant of Pseudomonas included. Forty children were of West African origin, 23 were dermatitis. Patient 1: a 23-year-old Asian woman presented white and a further 23 were of mixed or nonspecified ethnic- with a flare of atopic eczema. Both arms were affected by ity. All were under 14 years of age (mean age 5Æ8 years). All slate-grey dry flaky skin, accentuated over the antecubital fos- the patients had a clinical diagnosis of tinea capitis and were sae. Culture from skin swabs isolated Pseudomonas aeruginosa; the treated with oral itraconazole at doses ranging from 3 to 5 rash cleared with ciprofloxacin. Patch testing was negative mg kg–1 daily for a mean duration of 6Æ7 weeks (range 2– except for cobalt. DermolÒ 500 (Dermal, Hitchin, U.K.), an 12). Fungal scrapings were obtained from 54 patients, of antiseptic emollient, had been used in conjunction with topi- whom 34 were found to have positive cultures. Trichophyton cal steroids before the flare. Patients 2 and 3: a 5-year-old girl tonsurans was isolated in 24 patients, Microsporum canis and Tricho- with atopic eczema developed an erythematous flexural erup- phyton violaceum in three individuals each, and T. equium, T. tion with brown desquamation at its margins soon after start- rubrum, T. verrucosum and T. soudanense in single cases. A kerion ing Dermol 500. Swabs growing Pseudomonas were initially was identified in seven patients, all of whom responded to considered to be a contaminant. Her 3-year-old sister subse- treatment. Six patients had already received oral antifungal quently developed a similar rash. Both children were treated therapy including three who had completed at least 4 weeks with ciprofloxacin that cleared the rash, although it recurred of itraconazole. Of these, one was lost to follow-up, one was several times on stopping treatment. Both were using the same cured after a total of 12 weeks of treatment and one with M. supply of EpadermÒ (Medlock, Oldham, U.K.) that showed a canis subsequently required oral terbinafine. Seventeen patients heavy growth of Pseudomonas on culture. After new supplies of failed to attend after the initial visit. Of the remaining 69 emollient were provided, there was no further recurrence. patients, clinical cures (resolution of symptoms and signs) Patient 4: a 33-year-old Afro-Caribbean woman presented at were documented in 43 (62%) after 6 weeks or less of treat- 36 weeks of pregnancy with a widespread dark grey flexural ment, and 14 (20%) resolved after 6–12 weeks. An additional dermatitis with marked xerosis and fissuring. Skin swabs grew five (7%) were responding after 6 weeks of treatment but fur- Pseudomonas. The eruption resolved postpartum without the ther follow-up was unavailable, and four (6%) nonresponders need for antibiotics. Patient 5: a 64-year-old man was referred

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 41 with a grey scaly perianal eruption, having used topical anti- resistant to fusidic acid 32% in 2002 and 40% in 2007 had septics and antibiotics. Skin swabs confirmed a heavy growth been treated with topical fusidic acid before the development of Pseudomonas; the rash resolved following ciprofloxacin. of resistance. Conversely 64% had no documented exposure to Patient 6: an 80-year-old Afro-Caribbean man presented with the drug but nonetheless had resistant strains on their skin. penile oedema and scaly greyish erythema of the groin, axillae Just 9Æ5% of patients with documented use of topical fusidic and antecubital fossae. Swabs identified Pseudomonas. The rash acid on their skin grew sensitive strains of S. aureus. Lack of resolved with ciprofloxacin. In all cases there was a character- information on treatments given to the patients in the com- istic well-demarcated grey or brown discoloration of the flex- munity may partially explain these results. Staphylococcus aureus ural skin associated with xerosis and desquamation. These isolated from the skin of dermatology patients showed high features resembled those of dithranol staining, but no anthra- levels of fusidic acid resistance over the 5-year study period. lin-related derivatives or laxatives were implicated. Pseudomonas There were higher levels of resistance to antibiotics used topi- is a transient species of skin flora, mainly found at flexural cally than systemically. However the level of methicillin-resis- sites. It is normally kept in check by the dominant Gram-posi- tant S. aureus (MRSA) was significantly higher in 2007 tive cocci. Repeated use of bactericidal agents effective against compared with 2002. In addition 24% of the MRSA isolates in Gram-positive flora favours the establishment of Pseudomonas. In 2007 showed a resistance pattern compatible with commu- conclusion, a defective barrier function of the skin and super- nity-acquired MRSA. Knowledge of local antibiotic resistance infection with Pseudomonas may cause a distinctive dermatitis, patterns of S. aureus is useful in ensuring the best management which has not been described previously. of cutaneous infections in dermatology patients and to screen for possible community-acquired MRSA which may be in- creasing.

P-38 Antibiotic resistance patterns of Staphylococcus aureus isolates from dermatology patients over P-39 the past 5 years The presentation of HIV infection to dermatology: D. Bobak, I. Eltringham and R. Morris-Jones variations between African immigrants and King’s College Hospital, London, U.K. non-African patients Antibiotic resistance evolves through natural selection by ran- S.I. Goolamali, L. Schmulewitz, J. White, dom mutations and pressure from environmental factors such E.M. Higgins and J.D. Creamer as antibiotic exposure. Resistant genes can be transferred King’s College Hospital, London, U.K. between organisms via plasmid exchange leading to rapid A new diagnosis of HIV infection may be made following pre- aggregation of resistant strains on the skin. Multiresistant sentation with a variety of skin disorders. South East London strains of cutaneous Staphylococcus aureus are some of the most has one of the highest rates of HIV in Europe, due in part to a challenging infection issues currently facing hospital practition- large African immigrant population. Our study compared a ers. Management of cutaneous S. aureus infections with topical group of 13 patients born in sub-Saharan Africa with a group and/or systemic antibiotics requires up-to-date knowledge of 13 U.K.-born non-Africans all diagnosed with HIV in our about bacterial sensitivity and resistance patterns in the derma- dermatology clinic over a 6-year period from January 2002 to tology patient population. This study examined the antibiotic January 2008. In the African group, ages ranged from 21 to sensitivities of S. aureus isolates from skin swabs taken from 53 years (mean 34) with their countries of origin being Zim- dermatology patients seen in clinic. One hundred consecutive babwe (3), Uganda (3), Coˆte d’Ivoire (1), Congo (1), Eritrea isolates from January to June 2002 and 2007 were analysed. (1), Ghana (1), Nigeria (1), Somalia (1) and South Africa Patients had a range of underlying dermatological conditions; (1). All acquired HIV heterosexually and presented with a viral however the majority (55% in 2002, 54% in 2007) had ato- load (range < 50–217 000, mean 91 122) and CD4 count pic dermatitis. The age range of patients was 3 months to 86 (range 4–530, mean 147Æ6). The most common clinical pre- years, mean 28 years (2002) and 3 months to 92 years, mean sentation was of papular pruritic eruption (6 cases); the others 35 years (2007) (P > 0Æ05). High levels of resistant S. aureus were folliculitis (2), sebopsoriasis (1), urticaria + penile her- isolates were found for penicillin (85 and 90%), erythromycin petic ulcers (1), tinea facei + molluscum contagiosum (1), (28 and 17%) and fusidic acid (41 and 33%) in 2002 and vasculitis (1) and toxic epidermal necrolysis (TEN) (1). The 2007, respectively. Flucloxacillin resistance was seen in 2% of non-African group had ages ranging from 29 to 71 years isolates in 2002 and 17% in 2007 (P < 0Æ05). Fusidic acid (mean 41). All were born in the U.K. but two were of Carib- resistance was recorded in 50% of isolates from patients under bean and one of Greek ancestry. Eight of 13 cases were con- the age of 20 years of whom 94% had atopic dermatitis. Over tracted homosexually and presented with a higher viral load the age of 50 years 20% of patients had S. aureus isolates resis- (range < 50 to > 804 000, mean 254 798, P = 0Æ03) and tant to fusidic acid. The underlying dermatoses in the older higher CD4 count (range 4–780, mean 200Æ5, P = 0Æ54) com- age group were diverse. Of those patients who had S. aureus pared with the African group. The 13 non-African cases pre-

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 42 Bristol Cup Posters sented with warts (3), psoriasis (3), genital herpetic ulcers staging did not have their stage stated and three patients who (2), recurrent fungal infections (2), seborrhoeic dermatitis had stage 2B or 3B disease did not have CT staging docu- (1), folliculitis (1) and molluscum contagiosum (1). Conclu- mented. The pathology was reviewed in 38 patients by a mul- sions: African immigrants with HIV presented with a lower tidisciplinary team (MDT) of dermatopathologist and CD4 count, a statistically lower viral load and had acquired dermatologist. Of the 30 patients who did not have MDT HIV heterosexually. The range of skin disorders which led to review: 12 had stage 1A disease, 14 had higher stages requir- an investigation of suspected HIV infection differs in African ing MDT review, four did not have the stage documented. and non-African patients. Our African subgroup presented Pathology MDT review was dependent on the availability of more often with inflammatory dermatoses and significantly in specialist dermatopathology expertise and lack of histopathol- one case with a severe drug eruption (TEN). In two cases, the ogy details in the notes of 15 patients when initially referred. patients presented with dual pathology. In contrast, the non- In agreement with BAD guidelines, patients with stage 1A/1B African group presented with single pathologies commonly of received appropriate SDT; chemotherapy was reserved for an infective aetiology and of a less severe nature. A pruritic patients with stage 3 or 4 disease. Although a majority of papular eruption was a common presentation of HIV in the patients had appropriate staging investigations and pathology African immigrant patients but was not seen in the non-Afri- MDT review; this was not always clearly stated. We plan to can group. It is important to recognize that HIV infection in introduce a proforma for CTCL patients to improve future dermatology may present in different ways in African immi- documentation. grant and non-African patients

P-41 Audit of skin care in organ transplant recipients: P-40 how should dermatologists be involved in the Audit of cutaneous T-cell lymphoma management management of their skin? C. Angit and E.J. Parry L. Paul and C.C. Lyon Hope Hospital, Manchester, U.K. York Hospital, York, U.K. Joint BAD and U.K. Cutaneous Lymphoma Group guidelines Organ transplant recipients (OTR) are 100 times more likely for the management of cutaneous T-cell lymphoma (CTCL) to develop skin cancer compared with the general population were published in 2003 (Whittaker SJ, Marsden JR, Spittle M, (Berg D, Otley CC. Skin cancer in organ transplant recipients: Russell Jones R. Joint British Association of Dermatologists and epidemiology, pathogenesis, and management. J Am Acad Der- U.K. Cutaneous Lymphoma Group guidelines for the manage- matol 2002; 47: 1–17). Risk factors include ultraviolet expos- ment of primary cutaneous T-cell lymphomas. Br J Dermatol ure, duration and type of immunosuppression, and age at 2003; 149: 1095–107). We audited the initial management transplantation. The number of organ transplants being per- of patients referred to a tertiary referral CTCL clinic by retro- formed is increasing steadily in the U.K. each year, and sur- spective case note analysis, to compare our practice with BAD vival rates are improving with newer immunosuppressive guidelines. Between 2000 and 2006, 68 patients were diag- regimes. The OTR population is therefore increasing. The aim nosed with CTCL. Forty-one (60%) of the patients had myco- of this audit was to determine who was identifying skin dis- sis fungoides (MF), 22 (32%) CTCL variants and five (7%) ease in OTR, the numbers of skin cancers identified in this Se´zary syndrome. Of these, 39 (57%) were male. The age population and the role of dermatologists in screening. We range was 13–91 years (median 57). Twenty-nine (43%) reviewed the notes of 138 (72 male, 66 female) of 154 OTR patients presented at stage 1A, 16 (24%) at stage 1B, five in our hospital. Their age ranged from 18 to 80 years, with (7%) at stage 2B, two (3%) stage 3 and one of 68 at stage the mode being the 41–50 year category. The number of 4A. Staging was not stated in 15 (22%) patients. All 45 completed years since their organ transplant ranged from 1 to patients with documented stage 1 disease received appropriate 30 years. Fifty-five per cent of the patients had had their skin directed therapy (SDT): topical treatment (14), photo- organ transplant for 5 years or less. Of these, 41 patients were therapy (12), or a combination of both (19). In addition, referred for a dermatology opinion between January 2005 and four of stage 1 patients had acitretin (three with MF variants, July 2007. Referrals were made by the nephrologists in 30 one with MF and chronic lymphocytic leukaemia). Patients cases, the GP in five and other healthcare professionals in six with stage 2 disease (5) initially received photochemotherapy cases. The correct diagnosis was suggested by the referrer in (PUVA), topical treatment and PUVA, or PUVA with acitretin. 61% of patients. The dermatologists diagnosed 12 nonmel- Patients with stage 3 disease (2) received , fol- anoma skin cancers. Other conditions diagnosed included viral lowed by either PUVA or extracorporeal photopheresis. Eight warts (14), actinic keratosis/Bowen’s (6), basal cell papillo- of the patients had computed tomography (CT) staging. Of mas (3), molluscum contagiosum (2) and miscellaneous these, seven had stage 1 disease (three with MF variants), five benign conditions (10). One patient was referred for skin sur- had stage 2B, 3A or 4A disease, six patients who had had CT veillance due to a history of Bowen’s disease, but had no

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 43 active at review. Follow-up of these patients is (22%) had radial regression and 27 (23%) lesions showed by the dermatology department in 28 (68%) cases and GPs in vertical regression. The commonest site for relapse was local 13 (32%) cases. However many of the patients are at an early lymph nodes (57%), but some patients developed visceral dis- stage in their management. Follow-up of OTR seen in the ease as first relapse. At time of final information 47 patients dermatology department prior to January 2005 was by the GP (40%) were alive, including 27 women. Six patients (5%) in 77% of cases. Of these, 19 were re-referred to dermatol- were dead of unrelated causes and 56 (48%) were dead of ogy, 15 had a new problem and four had a flare or recurrence melanoma or unknown cause. Seven patients (6%) were lost of an old problem. In conclusion, skin disease in OTR is being to follow-up. While thin melanomas usually carry a good identified by other healthcare professionals, before being prognosis, a proportion will relapse and prove fatal. Sentinel referred to dermatologists. However it is unclear if complete node biopsy (SNB) is not usually undertaken in patients with cutaneous screening examinations of these at-risk patients is thin melanoma, although it could identify early disease in being done regularly. This could be undertaken by GPs, the draining lymph nodes. It is essential that efforts are made to transplant specialist team or specialist nurses, with prompt improve identification of the subgroup of patients with thin access to dermatology services if required. Alternatively, der- lesions likely to relapse at time of presentation. This would matologists could run specialist transplant clinics for skin sur- allow consideration of SNB or PET scanning. Immunohisto- veillance of OTR. Further work is needed to clarify the clinical chemical markers such as Ki67 are being developed to detect and cost effectiveness of specialist transplant screening clinics. cell division, and this type of approach may help highlight patients at risk of aggressive relapsing disease.

P-42 P-43 Thin relapsing melanomas: a 21-year review A survey of the management of Spitz naevi in S.J. Fraser, A.S. Krajewski* and V.R. Doherty children by BSPD members Department of Dermatology, Royal Infirmary of Edinburgh, U.K. and C. Feighery, J. McKenna and E.A. Bingham *Department of Pathology, Western General Hospital, Edinburgh, U.K. Royal Hospitals Trust, Belfast, U.K. Thin malignant melanomas (Breslow thickness (<1 mm) Last year we presented results of a survey on the management account for around three-quarters of all melanomas (Gimotty of Spitz naevi in children by Irish dermatologists (Feighery C, P, Elder D, Fraker D et al. Identification of high-risk patients McCarron M, Bingham EA. Survey on the management of among those diagnosed with thin cutaneous melanomas. J Clin Spitz naevi in children by Irish dermatologists. Br J Dermatol Oncol 2007; 25: 1129–34). Classically they have been consid- 2007; 157 (Suppl. 1): P-47). Worryingly out of the 31 ered to carry a good prognosis, with 5-year survival rates of replies, a significant proportion has seen a melanoma develop 98% quoted. There are, however, small numbers of patients at the site of what was clinically (10% or three people) or in whom the disease recurs, leading to morbidity and mortal- histologically (13% or four people) diagnosed as a Spitz nae- ity. Although various characteristics have been postulated to vus. As a result of this we went on to send out the same sur- increase this risk, no definitive factor has been found, so man- vey to all of the members of the British Society of Paediatric agement is difficult to tailor. Older men may be at higher risk, Dermatology (BSPD). We received 45 replies. Results showed and concerning pathological features include increased Bre- that 58% of respondents would biopsy what was clinically felt slow thickness and Clark level, ulceration, vertical growth to be a Spitz naevus in a child and 100% of these would do phase, regression and mitotic rate (Gimotty et al.). We used an excision biopsy and most would then NOT follow up. data collected by the Scottish Melanoma Group to review Eighteen per cent would follow up for a further year. Sixty- demographics and clinicopathological features of patients pre- two per cent of respondents did NOT feel that pigment would senting with thin malignant melanomas (MM) in the East of make a Spitz naevus in a child more suspicious. Of the doctors Scotland between 1979 and 1999. Patients with metastatic dis- who did not excise Spitz naevi in children the follow-up ease at presentation were excluded. A total of 1976 patients varied: some altered follow-up according to the history of the were recorded with thin MM. One hundred and sixteen lesion, some followed up for 1 year (38%), and others fol- patients (6%) developed relapse (recurrence or death); 56% of lowed up until the child was old enough for excision of the patients with relapse were female. Average age at presentation lesion. The majority of respondents when asked what their was 55Æ1 years (unavailable in three patients). The face, back expected end result was commented that they expected these and lower leg were the commonest sites of primary MM. The lesions to resolve with time or turn into typical benign naevi. average Breslow thickness was 0Æ58 mm (range 0Æ1–0Æ98). However many people did not answer as they ALWAYS excise Forty (34%) lesions were Clark level 2, 43 (37%) were level Spitz naevi in children. Eighty-two per cent felt that these 3, and 32 (28%) were level 4. Seventy-one lesions (61%) were benign lesions when directly asked, the remaining 15% were superficial spreading subtype and 31 (28%) were lentigo said they did not know if they were a benign or a malignant maligna melanoma. Seventeen (15%) exhibited ulceration, 26 entity. A similar response was noted among Irish dermatologists

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 44 Bristol Cup Posters with 87% responding that they felt Spitz naevi in children patients (52%) were discharged without further intervention. were benign. Two per cent (one person) of BSPD members This study confirms our view that in experienced hands tele- stated that they had seen a melanoma arise at a site of what dermatology may have a useful role in screening suspicious was originally felt clinically to be a Spitz naevus in a child and skin lesions and has proved invaluable as a management tool in similarly 2% (one person) stated that they had seen a mela- our catchment area to maintain central government directives. noma arise at a site of what was originally histologically felt No serious pathology was found to be missed although confir- to be a Spitz naevus. The treatment of Spitz naevi in children matory histology of all discharged patients was obviously not remains varied and controversial and while the majority feel possible. Future use of dermoscopic images should further that clinically obvious lesions are benign many people con- increase our accuracy and discharge rate. tinue to excise all lesions (Gelbard SN, Tripp JM, Marghoob AA et al. Management of Spitz naevi: a survey of dermatologists in the United States. J Am Acad Dermatol 2002; 47: 224–30). The optimal management of these lesions has not been ascertained but perhaps this survey will be helpful in stimulating discus- P-45 sion and in producing more uniform management policies. Dermoscopy and artificial intelligence for melanoma diagnosis: a systematic review S. Rajpara, P. Botello, A.D. Ormerod and J. Townend* Aberdeen Royal Infirmary and University of Aberdeen, Aberdeen, U.K. and *Department of Public Health, University of Aberdeen, Aberdeen, U.K. P-44 The most recent systematic review of literature on dermoscopy Rapid access skin cancer teledermatology 1986–2000 (Kittler H, Pehamberger H, Wolff K, Binder M. S.M. Halpern and L. Shall Diagnostic accuracy of dermoscopy. Lancet Oncol 2002; 3: Medway Maritime Hospital, Gillingham, U.K. 159–65) concluded that dermoscopy improves the diagnostic Telemedicine is widely used to aid diagnosis and manage accuracy for melanoma compared with the unaided eye. dermatology patients, either for reasons of distance from the A meta-analysis (Rosado B, Menzies S, Harbauer H et al. specialist, or managing demand within the contexts of achiev- Accuracy of computer diagnosis of melanoma: a quantitative ing government target criteria. We have historically used tele- meta-analysis. Arch Dermatol 2003; 139: 361–7) of computer dermatology flexibly to keep within waiting time targets diagnosis (artificial intelligence) of melanoma (2003) found despite having four consultants for a population of 850000. that the diagnostic accuracy of computer diagnosis was similar Referrals to the 2-week cancer screening clinic have been noted to that of human diagnosis and computer diagnosis is accurate to vary considerably between 20 and > 50 per week, tending under experimental conditions. Since these two reviews, there to peak during the summer months, which may strain clinic have been more than 400 studies published on dermoscopy capacity considerably. From July 2007 we have used our tele- and computer-aided diagnosis of melanocytic skin lesions. We dermatology system (E-ceptionist software), to screen a pro- conducted a systematic review to evaluate the diagnostic portion of 2-week referrals to ease the pressure on rapid-access accuracy of dermoscopy and artificial intelligence for mela- clinic space. A total of 17–20 patients per week were seen in a noma diagnosis and to compare the diagnostic accuracy of dif- specific Friday clinic by the teledermatology nurse specialist ferent dermoscopic algorithms. An extensive search of who took a standardized history and high-resolution close-up literature databases 1986–2007 was carried out to identify pri- images using a Nikon D70 camera. Information was down- mary studies, ongoing studies, abstracts and systematic loaded to a web-based server and two consultants with consid- reviews. The methodological quality of the studies was erable experience of teledermatology reporting, viewed and assessed as per the National Institute for Health and Clinical reported on the cases over that weekend to ensure that waiting Excellence (NICE) guidelines and the Standards for Reporting times were not breached. Between 20 July 2007 and 12 Octo- of Diagnosis Accuracy (STARD initiative). Funnel plots and ber 2007, 228 patients were assessed in this manner. Thirteen summary receiver operating characteristic (SROC) curves were lesions (6%) were diagnosed as probable malignant melanomas used to check for publication bias and threshold effect and all confirmed as such by histology following excision amongst the studies, respectively. A meta-analysis was carried within 2 weeks. Of eight (4%) suspected invasive squamous out and sensitivity, specificity, positive and negative likelihood carcinomas two were subsequently found to be keratoacan- ratios and diagnostic odds ratios were pooled, and forest plots thomas, one a basal cell carcinoma and one a leiomyosarcoma. were produced to illustrate variability between studies. Sensi- Forty-three patients (19%) were listed for less urgent surgery tivities and specificities of the different methods were com- including two dysplastic naevi (confirmed on histology) and pared using the binomial test and logs of likelihood ratios and 35 basal cell carcinomas (one of which proved to be an SCC). diagnostic odds ratios were compared using t-tests. Correc- Forty-four patients (19%) were recalled to clinic for face to tions for multiple comparisons were carried out using the face assessment with dermoscopy or diagnostic biopsy; in this Bonferroni method. From the 765 articles retrieved, 30 studies group one SCC was diagnosed and no melanomas. In total 119 were eligible for meta-analysis. Pooled sensitivity for artificial

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 45 intelligence was slightly higher than for dermoscopy (91% vs. study also raises the question of whether HHV-8 screening of 88%) (P = 0.076). Pooled specificity for dermoscopy was high-risk individuals pretransplant is indicated, and we are significantly better than artificial intelligence (86% vs. 79%) currently investigating this in our cohort. In seropositive indi- (P < 0.001). There was no significant difference between the viduals, risk may be reduced by more intensive clinical and different diagnostic algorithms. Dermoscopy and artificial virological surveillance and tailoring of post-transplant immu- intelligence did equally well for diagnosis of melanocytic skin nosuppressive regimens to include, for example, mTOR inhib- lesions. They have a complementary role for melanoma diag- itors, but this requires confirmation. With growing numbers nosis, with one providing high sensitivity and the other one of individuals from high-risk endemic areas on transplant high specificity. A prospective randomized controlled trial waiting lists and with the introduction of renal transplantation comparing dermoscopy and artificial intelligence should for HIV-positive individuals in some centres, the detection, provide more concrete evidence of their usefulness. prevention and management of post-transplant KS is likely to become an area of intense clinical need and research interest in the near future.

P-46 Kaposi’s sarcoma in organ transplant recipients: an increasing problem with implications for dermatological surveillance A. Gulati, D. Herath, J. McGregor, H. Dobbie,* P-47 R. Cerio, C. Proby and C. Harwood IgG antibasement membrane antibodies detected in Departments of Dermatology and Renal Medicine and Transplantation and patients with psoriasis *Institute of Pathology, Barts and the London NHS Trust, London, U.K. C. DeGiovanni, B. Bhogal*, E. Upjohn*, M. Allen*, Kaposi’s sarcoma (KS) is a multicentric spindle cell tumour of J.N.W.N. Barker* and R.W. Groves* probable endothelial cell lineage due to infection by human Dermatology Department, Brighton and Sussex University Hospitals NHS herpes virus 8 (HHV-8), and immunosuppressed organ trans- Trust, East Sussex, U.K. and *St John’s Institute of Dermatology, London, plant recipients (OTRs) are at up to 500-fold increased relative U.K. risk. However, there is considerable variation in disease preva- The autoimmune bullous disease, bullous pemphigoid (BP) lence, ranging from 0–0Æ13% in Northern European OTRs to has been associated with psoriasis, with 40 cases reported in 5Æ3% in Saudi Arabia, and this largely reflects geographic vari- the literature (Wilczek A, Sticherling M. Concomitant psoriasis ations in endemic HHV-8 infection. In the U.K., KS in most and bullous pemphigoid: coincidence or pathogenic relation- OTR cohorts is rarely seen. However, at our Institute, approxi- ship? Int J Dermatol 2006; 45: 1353–7). However the true nat- mately one-third of new OTRs are non-Caucasian, and KS is ure of this potential association is undetermined. To explore an increasing problem. In this study we retrospectively analy- this issue further we have collected 100 serum samples from sed clinicopathological features and outcomes of KS in our patients with psoriasis attending dermatology clinics at St cohort of >1000 OTRs under longitudinal surveillance John’s Institute of Dermatology and Brighton General Hospital. between 1987 and 2007. A total of 16 cases of KS were Ninety-four serum samples from age- and sex-matched con- observed, including eight (50%) in the past 5 years. None trols, without inflammatory skin disease, were also collected. were HIV positive. Nine (56%) were African, representing Indirect immunofluorescence on normal and split skin at titres over 20% of our African OTRs. Three (19%) were Afro-Carib- of 1 : 10 and 1 : 100, and enzyme-linked immunosorbent bean, two (12%) were U.K.-born white and there were single assay (ELISA) to BP180 were performed on each sample. Of cases in Mediterranean and Asian OTRs. The median duration the 100 psoriasis patients, 61 were male (mean age 49.3 of transplantation was 24 months. Skin involvement was years, range 21–86) and 39 were female (mean age 46.9 histologically confirmed in all 16 cases, with 10 (62%) affect- years, range 22–72). Fifty-six of the psoriasis patients were on ing lower limbs only and four (25%) legs and other sites. systemic medication (28 methotrexate, nine ciclosporin, six Limb oedema preceded skin involvement in 13/14 (93%) infliximab, four acitretin, four both methotrexate and ciclos- with leg KS. Visceral KS occurred in four (25%). Immunosup- porin, one hydroxyurea, one mycophenolate mofetil, one ada- pression was reduced in 11 cases (including four changed to limumab, one prednisolone and one etanercept). Seven of 100 the mTOR inhibitor rapamycin) and withdrawn in four cases. psoriasis samples were positive on immunofluorescence Three OTRs required chemotherapy and two radiotherapy. whereas 0/94 control samples were positive (P = 0.0087, Graft failure or death occurred as a direct consequence of KS Fisher’s exact test). Eight of 100 psoriasis samples were posi- in seven (44%) OTRs. This study has confirmed KS to be a tive on BP180 ELISA [mean 20 arbitrary units (AU), range significant problem in African OTRs in our Institute, often 10–49] whereas only 1/94 controls was positive on ELISA at occurring rapidly post-transplant, and resulting in considerable 10 AU (P = 0.022, Fisher’s exact test). Six psoriasis samples morbidity and death. It highlights the importance of assessing were positive on ELISA but had negative indirect immuno- OTRs from high-risk areas soon after transplantation and mon- fluorescence. Of the psoriasis samples that were positive with itoring closely for early signs of KS such as leg oedema. This immunofluorescence, only two samples were positive to

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 46 Bristol Cup Posters

BP180 on ELISA. Both of these samples showed high-titre formed; PASI and DLQI were documented in 90% of patients. (1:100) immunofluorescence staining. There did not appear to The response rates based on the NICE criteria for etanercept be an association between systemic treatment for psoriasis and and efalizumab were 85% and 57%, respectively. Based on the the presence of antibasement membrane antibodies. Our data BAD criteria, the response rates for etanercept, efalizumab and supports the concept of a true association between BP and infliximab were 73%, 57% and 91%, respectively. PASI 75 at psoriasis. Both diseases affect the basement membrane and week 12 was achieved in 48%, 43% and 82% of etanercept, alteration or disruption of the basement membrane in psoriasis efalizumab and infliximab patients, respectively. Only 28% of may lead to exposure of previously hidden autoantigens and etanercept patients had discontinued treatment after 24 weeks. development of autoantibodies. The discordance between the One efalizumab patient developed thrombocytopenic purpura immunofluorescence and ELISA data suggests that autoantibo- complicated by deep venous thrombosis and pulmonary dies other than BP180 may be of relevance in psoriasis-asso- embolism; and one etanercept patient developed a cerebral ciated BP and further studies are underway to explore this. vascular accident. In conclusion, NICE guidance was followed, patients were appropriately selected and there was no signifi- cant organ toxicity. However two adverse events occurred, indicating the continued vigilance necessary for this patient group and the need for the BAD Biologics Registry.

P-48 Audit of the use of biological agents for psoriasis N. Hashim, B. Dever and R.A.G. Parslew Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, P-49 Merseyside, U.K. Two years’ experience with adalimumab in severe, An audit was conducted to determine if biological interven- recalcitrant psoriasis tions are appropriately used in the treatment of adults with C. Ryan, B. Kirby, P. Collins and S.C.F. Rogers psoriasis based upon the National Institute for Health and St Vincent’s University Hospital, Dublin, Ireland Clinical Excellence (NICE) and British Association of Dermatol- Tumour necrosis factor-a inhibitors offer an effective treatment ogists (BAD) guidelines (Smith CH, Anstey AV, Barker JNWN for psoriasis in patients in whom other systemic agents are not et al. British Association of Dermatologists guidelines for the tolerated or have proved ineffective. We assessed the efficacy use of biological interventions in psoriasis 2005. Br J Dermatol and safety profile of adalimumab in patients with severe, recal- 2005; 153: 486–97), and also to evaluate agent efficacy and citrant psoriasis treated with adalimumab for a mean of 60Æ2 patient outcome. Data were extracted from the departmental weeks (24–102). We also studied the overlapping of other sys- biologics database in September 2007. There were 62 patients, temic treatments with adalimumab to prevent flaring of disease 52% female and 48% male, with a mean age of 48 years in patients with severe disease. A retrospective study was per- (range 25–79). Fifty-one patients (82%) fulfilled the NICE formed on 43 patients treated with adalimumab between Octo- eligibility criteria; however all met the requirements based on ber 2005 and January 2008. Patients were commenced on 40 the BAD guidelines. Eighteen patients (29%) also suffered mg adalimumab once weekly, or once fortnightly, as clinically with . Thirty-eight patients (61%) received indicated. Patients were reviewed at 8–12-week intervals for etanercept; 12 (19%) received efalizumab and 12 (19%) clinical response and adverse events. The therapeutic response received infliximab. Etanercept was the first choice used in 54 was assessed by the Psoriasis Area and Severity Index (PASI). patients (87%), efalizumab in seven patients (11%) and inflix- Forty patients (93%) had chronic plaque psoriasis, two (5%) imab in one patient (2%). All etanercept patients received 25 had palmoplantar pustular psoriasis and three (7%) acroderma- mg twice weekly dose. In one patient this was increased to 50 titis continua of Hallopeau. All had failed to respond to other mg twice weekly at week 12 due to uncontrolled arthritis. All systemic agents (mean of 3Æ9 agents) including other biologic efalizumab patients received the standard dose. For infliximab, therapies – infliximab in 15%, etanercept in 53% and 10 patients (83%) received 5 mg kg–1 8-weekly dose; one efalizumab in 9%. Fifteen patients had combined treatment had 5 mg kg–1 5-weekly dose and one had 6 mg kg–1 with another systemic agent at the onset of adalimumab treat- 8-weekly dose. For etanercept patients, 39% had monotherapy ment to prevent disease flaring. A 75% reduction in PASI rela- and 39% in combination with a reducing dose of ciclosporin. tive to baseline (PASI-75) was achieved in 83% at a mean of 18 Seventy-five per cent of efalizumab patients had monotherapy weeks and a 90% reduction in PASI (PASI-90) was achieved in and 75% of infliximab patients had concomitant methotrexate 63% at a mean of 22Æ5 weeks. Fifteen patients (35%) achieved therapy. Pretreatment Psoriasis Area and Severity Index (PASI) a PASI of zero at some point in their treatment. When the group and Dermatology Life Quality Index (DLQI) were documented treated with adalimumab only were compared with those who in 93% of patients while other baseline investigations were required overlapping treatment, a more rapid response was carried out in all patients. Tuberculin test was carried out in noted in the combination group, although efficacy was similar five patients and one was referred to the thoracic physician to between the groups at 72 weeks. Adalimumab was well toler- exclude sarcoidosis. All week 12 investigations were per- ated: 49% of patients experienced side-effects which were gen-

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 47 erally mild. Treatment was withdrawn in one patient for a commonly found KIR haplotype in Caucasians and contains severe injection site reaction and in another for pruritus. One the genes coding for multiple stimulatory KIRs (KIR2DL1, patient developed metastatic lung cancer while on treatment KIR2DL3, KIR2DL4, KIR3DL1, KIR3DL2, KIR3DL3) and the single and died. Another developed a squamous cell carcinoma on a inhibitory KIR (KIR2DS4) was found significantly less fre- leg. Infections accounted for 62% of side-effects but none was quently in ALD patients compared with controls (9% vs. 40%, serious. We found adalimumab to be highly effective in a group P < 0Æ001, OR 0Æ14). These data suggest that in our popula- of patients with severe, recalcitrant psoriasis, and to be well tion susceptibility to psoriasis is not predicated by possession tolerated. This is the first study to examine the overlapping of of particular KIR genotypes. Susceptibility to ALD is however other systemic treatment with adalimumab to prevent flaring of associated with possession of stimulatory KIRs. disease in patients with difficult psoriasis. We found this to be a safe and efficacious practice in our small group of patients. Declaration of financial interests: Two of the authors have acted as consultants and advisors for Merck Serono, Wyeth and Abbott Pharmaceuticals. P-51 Long-term efficacy and safety of infliximab in the treatment of patients affected by psoriasis and psoriatic arthritis M. Papoutsaki, M. Talamonti, A. Giunta, A. Costanzo and S. Chimenti P-50 Department of Dermatology, University of Rome ‘Tor Vergata’, Rome, Italy Investigation of killer cell immunoglobulin-like Psoriasis is a chronic disease and therefore effective therapies receptors in patients with psoriasis and patients with alcoholic liver disease without significant long-term toxicity are needed. Infliximab A.-M. Tobin, A. O’Connor,* S. Kelly,* M. Etherson,* is a chimeric antitumour necrosis factor (TNF)-a monoclonal S. Norris, D.G. Doherty* and B. Kirby IgG1 antibody that binds with high affinity and specificity to St Vincent’s University Hospital, Dublin, Ireland; *National University of TNF-a and neutralizes its biological activity. Currently it is Ireland Maynooth, Kildare, Ireland and St James’s Hospital, Dublin, Ireland approved for the treatment of chronic diseases characterized A consistent association between psoriasis and alcohol abuse by TNF-a-mediated inflammation such as rheumatoid arthritis, has been documented with several studies reporting that the Crohn’s disease, ankylosing spondylitis, ulcerative colitis, pso- incidence of psoriasis is increased among alcoholics compared riatic arthritis and psoriasis. We performed an open-label clin- –1 with controls. Susceptibility to psoriasis has been found to be ical trial with infliximab at a dosage of 5 mg kg infused at associated with possession of genes coding for isotypes of the weeks 0, 2 and 6, followed by a maintenance dose every 8 killer immunoglobulin-like receptors (KIR), which control weeks up to 3 years of treatment. A total number of 249 natural killer (NK) cell activity in a cohort of Japanese patients patients were treated (123 plaque-type psoriasis, 126 psoriatic with psoriasis (Suzuki Y, Hamamoto Y, Ogasawara Y et al. arthritis). The efficacy was evaluated at baseline, week 22, 1 Genetic polymorphisms of killer cell immunoglobulin-like year, 2 years, 3 years and 4 years, by calculating the Psoriasis receptors are associated with susceptibility to psoriasis vulga- Area and Severity Index (PASI) and the Health Assessment ris. J Invest Dermatol 2004; 122: 1133–6). Various combinations Questionnaire (HAQ). Infliximab was generally well tolerated of 15 different KIR isotypes (nine inhibitory and six stimula- and no serious adverse events were noted. At present, 161 tory) are encoded in humans and the stimulatory isotypes, patients have reached the 22nd week of treatment, 109 KIR2DS1 and KIR2DS5, appear to predispose these individuals to patients have reached 1 year, 64 patients have reached 2 years, developing psoriasis (Suzuki et al.). It is not known whether 15 patients 3 years, and six patients 4 years of continuous the development of psoriasis in alcoholic patients is due to treatment. Infliximab treatment was effective and considerably effects of alcohol or whether psoriasis and alcoholic liver dis- improved patients’ PASI score (82% PASI 75 at week 22 while ease (ALD) share common susceptibility factors. To address 83.4% maintained PASI 75 at 4 years) and quality of life, as this question, we have identified KIR genotypes in 39 patients assessed by the HAQ (100% improvement of the mean score with psoriasis, 46 patients with ALD and 42 healthy subjects already at week 22 and maintenance at 4 years). Interestingly, using polymerase chain reaction amplification of genomic although in a high percentage of patients treated the efficacy DNA with sequence-specific oligonucleotide primers. None of was maintained during the years of treatment, in a significant the KIR genes or haplotypes was associated with psoriasis in number of them a mild recurrence, either of the psoriatic le- our population of Caucasian patients contrary to the finding in sions or of the pain symptoms occurred, approximately 6 the Japanese cohort. Compared with controls, however, signif- weeks after their latest infusion. Nevertheless, these relapses icantly higher frequencies of ALD patients possessed the stim- were transitory and the drug regained efficacy through the ulatory KIRs, KIR2DS2 [67% vs. 43%, P < 0Æ025, odds ratio continuation of the treatment while the symptoms were well (OR) 2Æ8] and KIR2DS5 (67% vs. 29%, P < 0Æ0005, OR 5Æ2), controlled, either by shortening the interval between infusions and the inhibitory isotype KIR2DL5 (74% vs. 43%, P < 0Æ001, or by administering a low dose of methotrexate for a short OR 3Æ8). Furthermore, the AA1 haplotype, which is the most period of time. Our experience indicates that infliximab is an

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 48 Bristol Cup Posters effective and safe therapy for the long-term treatment of pso- members and partners. Br J Dermatol 2007; 156: 1245–50). A riasis and may have a lower incidence of side-effects than tra- measurement tool, the Psoriasis Family Index (PFI), is being ditional systemic antipsoriatic therapies. developed to measure this secondary impact (Eghlileb AM, Basra MKA, Finlay AY. Initial validation and refinement of Pso- riasis Family Index (PFI-20). Br J Dermatol 2007; 157 (Suppl. 1): 54). The aim of this study was to examine the psychomet- P-52 ric properties of the 15 questions version (PFI-15) of the PFI. To evaluate the therapeutic effect of mesalazine Factor analysis was conducted to examine the underlying in cutaneous psoriasis: an open prospective trial structure of the PFI-15, reliability was assessed from the in- R. Jain ternal consistency of the items, and correlations were made Ima Hospital, Chandigarh, India between PFI-15 scores from a 1–2-week test–retest study. Twenty-one patients (18 men and three women aged 19–62 Validity was assessed from correlation between the PFI-15 and years) were included in the open prospective study. Eighteen Dermatology Life Quality Index (DLQI), Psoriasis Disability had moderate to severe stable plaque-type psoriasis, two had Index (PDI) and Psoriasis Area and Severity Index (PASI). the erythrodermic form and one had guttate exacerbation at Forty-six participants were recruited: 16 were family members the start of therapy. Pregnant women were excluded. All and 30 were partners (M = 11, F = 35, mean age 48 years). patients underwent a pretreatment evaluation of the Psoriasis Factor analysis of the 15 items of the PFI-15 questionnaire Area and Severity Index (PASI) and laboratory evaluations revealed four factors which explained 68% of the common including haemogram, renal parameters (urea and creatinine), variance. The PFI-15 demonstrated high internal consistency: liver function tests, chest X-ray, blood sugar, serum amylase Cronbach’s a = 0Æ86. Item to total correlation for all items and urine examination. The PASI was repeated at 4 weeks and was > 0Æ20 and ranged from 0Æ32 to 0Æ65. Test–retest analysis then at 8 weeks of treatment by two different investigators. demonstrated an intraclass correlation coefficient of 0Æ93. The Mesalazine was started at an oral dose of 400 mg twice daily sensitivity of the PFI-15 to change was shown by change in for 3 days, which was increased to 400 mg three times daily the family members’ or partners’ PFI-15 scores in cases where for 4 weeks and extended to 8 weeks depending upon the the patients’ clinical condition either improved or worsened. response. Nineteen of 21 patients completed the treatment. The convergent validity of the questionnaire was assessed by The baseline PASI score ranged from 3Æ3 to 29Æ8 (mean 16Æ2). testing the correlation of the PFI-15 with the DLQI, PDI and In 17 of 19 patients the PASI decreased at the end of 8 weeks PASI; there was strong correlation between families’ PFI-15 of therapy. Two patients (one with erythroderma and the scores and patients’ DLQI (r = 0Æ54, P < 0Æ01) and PDI scores other with guttate exacerbation) did not show any decline. (r = 0Æ59, P < 0Æ01). There was also a moderate correlation The PASI score at the 8th week decreased by 40–75% in seven between families’ PFI-15 scores and patients’ PASI scores (r = patients, by 25–39% in six patients and by less than 25% in 0Æ43, P < 0Æ01). This evidence of PFI-15 reliability and valid- four patients. Overall the mean baseline PASI of 16Æ2 fell to ity is an essential first step in establishing this measure as an 13Æ2 (P < 0Æ01) at 4 weeks and 10Æ1 (P < 0Æ01) at 8 weeks. additional outcome instrument for assessing quality of life of The median baseline PASI of 16Æ6 fell to 12Æ9 at 4 weeks and family members or partners of patients with psoriasis. 8Æ4 at 8 weeks. The decrease in median value of the PASI score at 4 and 8 weeks was also statistically significant. None of the patients reported any major side-effects except nausea in three (16%). No alteration in laboratory values was seen in any of the patients. The therapeutic results seen with mesal- P-54 azine in our patients are similar to those seen with newer The impact of skin diseases on the parents and drugs. Mesalazine therapy is relatively free from side-effects. spouses/partners of the patients: a descriptive We believe that mesalazine certainly needs to be tried further analysis in a randomized double-blind controlled fashion. M. Basra and A.Y. Finlay Department of Dermatology, Cardiff University School of Medicine, Cardiff, U.K. Skin diseases can have a pervasive effect not only on the qual- P-53 ity of life (QoL) of the patients but also on the QoL of their Further validation of Psoriasis Family Index (PFI-15): family members such as parents (Lawson V, Lewis-Jones MS, a new measure to assess the secondary impact of Finlay AY et al. The family impact of childhood atopic derma- psoriasis on family members titis: the Dermatitis Family Impact Questionnaire. Br J Dermatol A.M. Eghlileb and A.Y. Finlay 1998; 138: 107–13) and spouses/partners (Richards HL, For- Dermatology Department, Cardiff University, Cardiff, U.K. tune DG, Chong S et al. Divergent beliefs about psoriasis are Psoriasis has a major impact on the lives of family members associated with increased psychological distress. J Invest Dermatol and partners of patients (Eghlileb AM, Davies EEG, Finlay AY. 2004; 123: 49–56). The aim of this study was to compare Psoriasis has a major secondary impact on the lives of family two groups of patients’ family members, i.e. parents and

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 49 spouses/partners with regard to their experiences with from the doctor that day. The questionnaire was filled by patients’ skin diseases. These data are part of a larger study to patients immediately before the consultation. The doctor develop a new QoL instrument for dermatology patients’ fam- checked the patient’s written response towards the end of the ily members (Basra MKA, Sue-Ho R, Finlay AY. The Family consultation. A second questionnaire was given after the con- Dermatology Life Quality Index: measuring the secondary sultation to elicit the level of satisfaction with the consultation. impact of skin disease. Br J Dermatol 2007; 156: 528–38). Adult Three hundred patients (male 126 and female 174, age range family members accompanying the patients to the dermatol- 1–89 years, 208 new and 92 review patients) completed the ogy outpatient clinic of a secondary referral centre were survey. Results are divided into two groups of patient fears requested to complete the Family Dermatology Life Quality and patient expectation. Patient fears: There were 11 different Index (FDLQI) (Basra et al.). It contains 10 items about differ- categories of fears based on words used by the patients. The ent aspects of family’s QoL which might be affected by commonest categories were future deterioration (31%), fear patients’ skin disease. Family members were divided into two of cancer (30%), persistence of condition (24%), symptoms groups: parents and spouses or partners. Nonparametric statis- (21%) and unsightly appearance (15%). Fear of the unknown, tics were used to examine differences between the two infectivity and fear of scarring were the lowest scoring fears. groups. A total of 121 adult family members (parents 48Æ8%, Fifty per cent of patients had a single fear about their skin spouses/partners 51Æ2%) of patients suffering from different condition while 34% had multiple fears; 13% of patients skin diseases (n = 42) completed the FDLQI. The mean age of reported ‘no fears’ relating to their skin condition. Multiple parents was 43Æ2 years (± 8Æ4) and of spouses/partners was fears were common in patients with inflammatory dermatoses 57Æ8 years (± 18Æ3, P < 0Æ001). The patients in the ‘parent (25%) compared with patients attending for lesion check-up group’ were younger (mean age 15Æ2 years, ± 6Æ8) compared (7%). Multiple fears occurred in 25% of new patients and in with patients in the ‘spouses/partner group’ (mean age 57Æ9 52% of review patients. The doctor was unaware of the years, ± 18Æ2, P < 0Æ001) with a shorter mean duration of patient’s fears in 34% of consultations. Patient expectations: There skin disease (parent group 5Æ2 years, spouses/partner group were twelve categories of response in terms of patient’s 10Æ5 years, P = 0Æ014). The most frequent skin disease in the expectation. One-third had multiple expectations. The com- parent group was acne (n = 20) followed by atopic eczema monest expectation was treatment/control of their skin condi- (n = 13) and psoriasis (n = 6). On the other hand the most tion (48%), reassurance and advice (42%) and seeking frequent disease seen in the spouse/partner group was psoria- diagnosis (21%). The desire for cure (eradication) was men- sis (n = 12), followed by atopic eczema (n = 7), solar kerato- tioned by 12%. Thirty-five (11%) patients have unrealistic ex- sis (n = 6) and basal cell carcinoma (n = 4). The mean FDLQI pectations. Consultation satisfaction: 93% were satisfied with the score of parents was 9Æ7 (± 6Æ3) while for spouses/partners consultation. However, 5% felt no change and 1% had unre- the mean FDLQI score was 7Æ4 (± 6Æ5, P = 0Æ028). The results solved fears after the consultation. In conclusion, patients have of this study have demonstrated that in a general dermatology a wide range of fears about their skin condition and these are secondary care clinic population, the overall impairment expe- often multiple and unexpressed. If these fears are not elicited, rienced by the parents of patients with skin disease is greater expressed by the patients and dealt with in the consultation, compared with that experienced by spouses or partners of the they remain and possibly may multiply as more review patients. This might be explained by the relatively younger patients had multiple fears than new patients. Patients and hence more dependent age of patients in the former acknowledged and appreciated that these questions were asked group as well as better coping capabilities in the latter group. as it had helped them express their fears and expectations.

P-55 P-56 Patients’ fears and expectations: exploring the Influences on clinical decision-making in hidden agenda in the dermatology consultation dermatology outpatient clinics K. Ahmad and B.C.P. Ramsay F.M. Hajjaj, M. Basra, S. Salek* and A.Y. Finlay Midwestern Regional Hospital Limerick, Limerick, Ireland Departments of Dermatology and *Pharmacology, Cardiff University, Cardiff, Patients have fears and therapeutic expectations in relation to U.K. their skin condition. These aspects maybe ignored or over- Decision-making lies at the very heart of clinical dermatology. looked in a busy clinic, whilst the dermatologist focuses on The literature regarding this subject in nondermatological spe- diagnosis and treatment. We therefore, explored the impact of cialties is expanding (Cook SA, Rosser R, James MI et al. Fac- skin disease from the perspective of patients’ fears, level of tors influencing surgeons’ decisions in elective cosmetic understanding of their condition and their therapeutic expect- surgery consultations. Med Decis Making 2007; 27: 311–20). ations. Patients were given three open-ended questions aimed However, in dermatology little is known about the process of at eliciting their level of understanding about their skin condi- decision-making and the influences on decisions (Katugampola tion, any associated fears or worries and what they wanted RP, Hongbo Y, Finlay AY. Clinical management decisions are

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 50 Bristol Cup Posters related to the impact of psoriasis on patient-rated quality of greater impact on QoL. The mean DLQI on the day of clinic life. Br J Dermatol 2005; 152: 1256–62). The aim of this study was 6Æ5 (SEM 1Æ02, n = 50) and at height of disease was 17Æ6 was to explore the management decisions made in dermatol- (SEM 1Æ34, n = 50). The mean difference in the two values ogy outpatient clinics and the main factors influencing these for each patient was 11 (P = 0Æ001). Seventy-eight per cent of decisions. The investigator (F.M.H.) observed the consultations patients had a higher DLQI score at the height of the disease of patients at a university hospital dermatology outpatient indicating a marked beneficial effect of treatment on QoL. clinic. Verbatim notes were taken of the conversations DLQI scores at the height of disease were comparable in both between the clinicians and the patients. Inferences were drawn PV (mean 18) and PF (mean 15) groups suggesting that both regarding different management decisions taken during these conditions have a similar impact on QoL. QoL is clearly consultations and the possible factors influencing these deci- severely impaired in pemphigus. Treatment seems to have a sions. A total of 168 consultations (consultants 60%, specialist marked beneficial effect on health quality. However, despite registrars 31%, clinical lecturers 5%, clinical nurse specialists treatment many patients have continued impaired QoL sug- 4%) were observed. The duration of each consultation ranged gesting that improved patient management protocols are from 2 to 37 min (mean 11Æ5 min; SD 6Æ8) with a significant required. difference between the consultation times for different clini- cians (P < 0Æ0001); the mean consultation time for specialist registrars was 15.5 min (SD 7Æ4) while for consultants it was 8.9 min (SD 4Æ8). Clinical decisions made during the consult- ations were classified into 21 main categories; the most fre- P-58 quently made decisions included: carrying out laboratory The contribution of skin cancer assessment and investigations (27%), starting new topical treatment (22%), treatment to total dermatology department activity: discharge to primary care (17%), renewal of systemic medica- a prospective region-wide audit from the U.K. tion (16%) and follow-up (11%). A total of 18 factors influ- M. Singh, E. McMullen, S. Ogden, S. Raynor* and encing these clinical decisions were identified. The R.J.G. Chalmers commonest were: clinical guidelines (23%), improvement of Hope Hospital, Manchester, U.K. and *Greater Manchester & Cheshire skin condition (17%), deterioration of skin condition (16%), Cancer Network, Manchester, U.K. side-effect of medication (8%), patients’ preferences (7%) and Skin cancer (both melanoma and nonmelanoma) accounts for quality of life issues (7%). Decision-making is an important 20% of all cancers diagnosed in the U.K. Dermatologists play aspect of clinical practice. The complexity of this process a lead role in diagnosing and managing suspected cases but revealed by this initial study highlights the need for more supporting evidence is gathered mostly from cancer registries. structured research in this area. There are no prospective data published looking at the exact nature of the skin cancer workload in the U.K. An audit to investigate what proportion of a dermatologist’s clinical work is committed to managing skin cancer was conducted in 12 dermatology departments serving a total population of over 3 P-57 million people. Prospective data on all clinical activity (includ- Quality of life (QoL) is significantly impaired in ing nurse-led and phototherapy) over a 4-week period in patients with pemphigus November 2006 were collected by proforma. The information M. Saha, S. Grieve and R.W. Groves included the number of attendances, the number of melano- St John’s Institute of Dermatology, King’s College London, London, U.K. mas and nonmelanoma skin cancers suspected by a dermatolo- Pemphigus is a rare, potentially fatal, autoimmune blistering gist, the percentage dealt with ‘one-stop’, those biopsied or disorder. The condition has a chronic course and can lead to managed ‘in house’, and those referred on to surgical special- many years of recurrent painful blisters and erosions on the ties, oncology, Mohs’ surgery or photodynamic therapy skin and mucous membranes. However, the impact on quality (PDT). The proportion of this work attributable to HSC205 of life (QoL) for pemphigus patients has not been studied in urgent 2-week wait referrals was reviewed. Information on detail. The aim of this study was to evaluate a well-studied 11 164 attendances from 903 clinical sessions was analysed: health-related quality of life index (Dermatology Life Quality of the 3951 new patients seen (representing 35% of total Index, DLQI) (Finlay AY. Quality of life assessments in derma- attendances), 9% (n = 355) had been referred under HSC205 tology. Semin Cutan Med Surg 1998; 17: 291–6) in individuals rules. The nonattendance rate was lower in the HSC205 group with pemphigus vulgaris (PV) and pemphigus foliaceus (PF). (6% vs. 12% overall). Twenty-seven per cent of patients Fifty patients with pemphigus (PV 37, PF 13) with stable dis- referred by HSC205 were thought clinically to have melanoma ease on systemic immunosuppression were recruited for the (n = 50) or squamous cell carcinoma (SCC) (n = 39), and study. Patients were asked to complete a DLQI questionnaire 14% basal cell carcinoma (BCC) (n = 48). A total of 471 based on their disease severity on the clinic day and retro- patients suspected by a dermatologist to have skin cancer were spectively at the height of disease activity. The DLQI score var- referred by other routes: melanoma (n = 41) or SCC (n = 88) ies from 0 to 30 with an increased score associated with a in 21% and BCC (n = 342) in a further 56%. Twenty-four per

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 51 cent of all new referrals were for suspected skin cancer, PION; 245 were included in the present analysis. Mean Psoria- among which 91 suspected melanoma and 517 suspected sis Area and Severity Index (PASI) scores at baseline were nonmelanoma cancers were found. Overall, 41% of HSC205 20Æ2, 19Æ4, and 19Æ2 for the ADA, MTX and placebo groups, referrals and 15% of other referrals were investigated or trea- respectively. Eighty per cent of ADA patients reached a ‡ PASI ted as skin cancer by a dermatologist. Of these cases, 91% 75 response at week 16 vs. 36% of MTX and 19% of placebo were either biopsied or treated in house with 9% given a patients (P < 0Æ001, ADA vs. MTX or placebo). More patients ‘one-stop’ service. Forty-one patients were referred for Mohs’ in the ADA group entered the study with very large or surgery and five for PDT. Fourteen per cent (n = 96) of extremely large effects on HRQOL (55% vs. 42% for MTX and patients were referred on to other disciplines (76 for plastic 48% for placebo). At week 16, 63% patients in the ADA surgery, 10 for radiotherapy, one to medical oncology and group had achieved a DLQI score of 0–1, indicating no (nega- nine elsewhere). Follow-up of skin cancer patients accounted tive) effect on HRQOL vs. 46% for MTX and 22% for placebo. for 10% of review patients seen during the 4-week audit per- In addition, by week 16, only 3% of ADA patients reported iod. This is the first prospective study to demonstrate that skin DLQI scores indicating their psoriasis had very/extremely large cancer assessment and treatment accounts for almost a quarter effects on HRQOL vs. 10% in the MTX group and 26% in of the clinical workload of U.K. dermatology departments and the placebo group. Dermatology-specific quality of life is an that dermatologists manage the majority of skin cancers important component of the overall management of patients referred to them (86% in this audit). with psoriasis. In the CHAMPION trial, ADA was associated with greater improvement in DLQI than both MTX and placebo. This study was sponsored by Abbott Laboratories. Two authors were investigators in the study, and two authors are employees of Abbott. P-59 Reducing the impact of psoriasis on patient quality of life with adalimumab: results from the CHAMPION P-60 study Vulvodynia: a delayed diagnosis J.-H. Saurat, J.-P. Ortonne,* K. Unnebrink and T. Cheung and F.M. Lewis* M. McIlraith Solihull Hospital, Solihull, U.K. and *King Edward VII Hospital, Windsor, Hoˆpital Cantonal Universitaire, Geneva, Switzerland; *Hoˆpital L’Archet, Nice, U.K. France and Abbott GmbH & Co. KG, Ludwigshafen, Germany Vulvodynia is defined as ‘vulval pain in the absence of rele- This analysis evaluated the impact of adalimumab (ADA), a vant, visible physical findings, or a specific, clinically identifi- fully human, antitumour necrosis factor monoclonal antibody, able neurological disorder’ (Moyal-Barracco M, Lynch PJ. versus methotrexate (MTX) or placebo on patient health- 2003 ISSVD terminology and classification of vulvodynia: a related quality of life (HRQOL) in the treatment of moderate historical perspective. J Reprod Med 2004; 49: 772–7). It may to severe psoriasis. Chronic plaque psoriasis is associated with be generalized or localized, provoked or unprovoked. Patients impairment in dermatology-specific HRQOL, as measured by often undergo multiple investigations and treatments before the Dermatology Life Quality Index (DLQI). DLQI scores range the diagnosis is made. We have evaluated the delay between from 0 to 30 and Finlay and Khan (Finlay AY, Khan GK. Der- the onset of symptoms and a final diagnosis of vulvodynia matology Life Quality Index (DLQI) – a simple practical mea- and compared this with a range of other common vulval con- sure for routine clinical use. Clin Exp Derm 1994; 19: 210–16) ditions. The notes of 182 new patients seen in a vulval clinic correlated different score ranges with the magnitude of effect were reviewed. The final diagnoses were classed into the fol- on patient HRQOL: scores of 0–1 = no effect; 2–5 = small lowing groups: vulvodynia, lichen sclerosus, lichen planus and effect; 6–10 = moderate effect; 11–20 = very large effect; eczema/lichen simplex. Patients with other diagnoses were and 21–30 = extremely large effect. In this post-hoc subanalysis, excluded. The time that had elapsed from the onset of symp- the effects of ADA, MTX and placebo in reducing the magni- toms to the diagnosis being made was recorded. Twenty-one tude of the effect of psoriasis on patient HRQOL were com- patients (12%) were diagnosed with vulvodynia, 77 patients pared. Patients with moderate to severe plaque psoriasis who (42%) had lichen sclerosus, 61 (34%) had eczema/lichen enrolled in CHAMPION, a 16-week, phase III, double-blind, simplex and 23 (13%) had vulval lichen planus. The mean double-dummy trial, were randomized to one of three treat- delay between onset of symptoms and diagnosis was as fol- ment arms: (i) ADA (80 mg subcutaneously at week 0 and 40 lows: vulvodynia 7Æ21 years, lichen sclerosus 5Æ94 years, mg every other week from weeks 1–15); (ii) oral MTX, start- eczema/lichen simplex 4Æ19 years and lichen planus 4Æ09 ing at 7Æ5 mg per week and increasing, as tolerated/indicated, years. This study highlights the significant delay for many to a maximum dosage of 25 mg per week; or (iii) placebo. patients presenting with vulval symptoms. However, the delay Patients with DLQI data at both baseline and week 16 were for those with vulvodynia is almost twice that of other inflam- included in this subgroup analysis, which also examined matory conditions. Further education of primary care phys- changes in percentages of patients in each DLQI score range icians and those in other specialities where these women may during the study. A total of 271 patients enrolled in CHAM- present is vital in improving their care.

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P-61 P-62 Optimizing surgical resources in secondary care: a Audit of waiting times for assessment and treatment joint venture with the primary care trust of melanomas and squamous cell carcinomas in a T. Joseph, S. Jain and C.J.M. Stephens regional dermatology department Poole Hospital NHS Trust, Poole, U.K. C. Groves, M. Walsh and O.M. Dolan Skin surgery remains an important aspect of dermatological Belfast City Hospital, Belfast, U.K. services. The incidence of skin cancer in the South Coast is New government-set waiting time targets for cancers have one of the highest in the U.K. Sixty per cent of referrals to the been introduced in Northern Ireland over 2007. These consist Dermatology Department at our hospital are for suspected skin of three targets: target 1 – 31-day maximum wait from refer- cancer. Since the implementation of the 2005 Department of ral to decision to treat; target 2 – 31-day maximum wait from Health NHS cancer plan for a maximum 31-day wait from decision to treat and first definitive treatment; target 3 – 62- diagnosis to first treatment, there has been increasing pressure day maximum wait between referral and first treatment. With on surgical resources. To optimize the use of our limited reference to skin cancers, these targets apply to melanomas resources it has been necessary to be selective in the type of and squamous cell carcinomas (SCCs) with exclusion of basal surgical procedures being performed in secondary care. In cell carcinomas and Bowen’s disease. Any possible melanoma 2001, a prospective audit conducted over a 3-month period or SCC is to be ‘red-flagged’ by the GP. The aim of our audit in the Department of Dermatology showed that a significant was to assess if the three target periods had been achieved in proportion of our surgical work (34%) was for benign the period 1 February 2006 to 1 February 2007. All new lesions. Following this, with the support of the primary care diagnoses of malignant melanoma, lentigo maligna and SCC trust (PCT), a limited list for referrals was drawn up in line from 1 February 2006 to 1 February 2007 were identified by with many other dermatology departments in the country to the histopathology department. All charts were reviewed and discourage referral of benign lesions/cosmetic surgery to sec- the above time periods were determined. In addition a num- ondary care, and distributed jointly by the Dermatology ber of other factors were considered: referral source; ability of Department and PCT to all general practitioners. Any inappro- the referrer to grade malignant potential; adequacy of infor- priate referrals were sent back. In 2007 a prospective 3-month mation on referral letters; patients requiring referral to other re-audit was done to assess the impact of the limited list. Dur- specialties; and referral to diagnostics. Forty-three diagnoses of ing this time, 372 procedures were performed, of which 52 melanomas and 44 SCCs were made over this time period. were diagnostic biopsies of rashes. Of the remaining 320 pro- Thirty-three per cent of SCCs and 33% of melanomas met tar- cedures, 286 (89%) were for malignant/premalignant condi- get 1; 54% of SCCs and 71% of melanomas achieved target 2 tions – malignant melanomas 14, squamous cell carcinomas with 38% of SCCs and 34% of melanomas attaining target 3. 35, basal cell carcinomas 119, suspicious moles (29 histo- More than 50% of SCCs and 75% of melanomas were referred logically atypical, 13 histologically benign), solar keratosis 61, by general practitioners. Fifty-six per cent of SCC referrals Bowen’s disease 7, keratoacanthomas 4, lentigines 2 and pyo- were specified as urgent with only 24% marked specifically as genic granulomas 2. This study shows that nearly 90% of our a potential SCC; 56% of melanoma referrals were labelled work is now skin cancer surgery. The audit highlights that the urgent with only 20% marked as potential melanomas; 50% actions following our previous audit have been successfully of SCCs and 40% melanomas were referred to plastics, oncol- implemented. Avoiding nonessential surgery is important if ogy or both; 15% of melanomas required diagnostic services. we are to make the best use of secondary care surgical exper- We envisage that attaining the above targets will be problem- tise in the face of limited resources. Having stringent agreed atic. We identified problems with specificity and accuracy of referral protocols/guidelines can have a major impact on the referral letters along with delays in treatment associated with type of surgical work done in secondary care but requires those patients referred to other specialties. This may, in part, close cooperation with primary care. This change in practice be attributed to an absence of a multidisciplinary meeting has also gone some way towards moving appropriate derma- between specialties. Also, a number of pitfalls of the 31-day tological work back to the community, now a focus of the target were identified – capacity in clinics or theatres is a con- ‘Care closer to home’ NHS plans. cern along with the displacement of other urgent skin prob- lems. A large increase in the number of benign referrals is anticipated as any referrals red flagged by a GP will automati- cally be given an urgent referral.

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P-63 P-64 Ward referrals to dermatology: a regional audit in Cellulitis is best managed by a dedicated Merseyside dermatology clinic: improved diagnosis K. Panting, C. Love* and J. Ellison* and reduced inpatient bed use Broadgreen Hospital, Liverpool, Merseyside, U.K. and *Whiston Hospital, C. Wingfield, N. Levell and J. Garioch Prescot, Merseyside, U.K. Norfolk and Norwich University Hospital, Norwich, U.K. A regional audit was carried out over a 3-month period of all Cellulitis of the lower leg is often secondary to underlying ward referrals to the dermatology teams in three hospitals skin disease and accounts for 2–3% of hospital admissions. including direct referrals to dermatology specialist nurses in Previous studies show the average inpatient stay is 8.2 days one of these hospitals. The main aims of the audit were to with 25–50% suffering recurrent episodes (Department of establish the ward referral workload, to assess appropriateness Health Hospital Episode Statistics, 2002–3. Available at: of referrals and how they were made, and to identify how http://www.hesonline.nhs.uk/Ease/servlet/ContentServer?site- quickly patients were seen. A pre-designed questionnaire was ID=1937&categoryID=202 [last accessed 15 February 2008]). completed at the time of review for all patients and data were The Clinical Resource Efficiency Support Team recommended analysed using Microsoft Access. A total of 135 ward referrals (2005) that each hospital trust establish processes to promote were seen during the 3-month period [33 were seen at one of and implement recommended cellulitis management (CREST. the main teaching hospitals, 14 were seen by specialist nurses Guidelines on the management of cellulitis in adults. Clinical at one of the district general hospitals (DGH) and a further 35 Resource Efficiency Support Team, 2005. Available at: http:// and 53 patients were seen at two further DGHs]. Over 50% of www.crestni.org.uk/publications/cellulitis/cellulitis-guide.pdf referrals to doctors were made via a written proforma. These [last accessed 15 February 2008]). Patients with suspected cel- tended to have poor legibility and were often initiated by lulitis were previously managed in our hospital by the casualty junior members of the medical team. Over 50% of Specialist department or the emergency assessment unit–medicine Nurse referrals were verbal and almost 80% of these were ini- (EAUM). Only occasional patients were referred on to the tiated by junior medical or nursing staff, for example for leg dermatology department. It was suspected that the correct ulcers or Doppler assessment. As a region the audit high- diagnosis and underlying skin disorders were not always iden- lighted that ward referrals were seen quickly, with over 50% tified, causing unnecessary treatment and increasing the risk of of patients being seen within 48 h, and the majority of these recurrent episodes. A dermatology lower limb cellulitis same- being seen within 24 h. Most referrals were seen by a Special- day diagnostic service was set up, with access to community ist Registrar or Staff Grade and it was deemed that at least intravenous therapy. Patients with signs of septic shock were 50% of cases were necessary inpatient referrals. Less than 50% excluded. Audit was carried out on 20 consecutive patients of referrals had a suggested diagnosis written on the form. with suspected lower limb cellulitis triaged to this service When a diagnosis was suggested it tended to be correct; how- from EAUM. We assessed the resources required, appropriate- ever very few patients had any treatment initiated prior to ness of referrals, diagnosis, patient management/treatment referral. The majority of cases seen were severe eczema, psori- and saving of bed days. Fifteen patients had cellulitis – other asis, vasculitis and cellulitis. Around 60% of patients had been diagnoses included infected, stasis or contact eczema and ery- admitted for nondermatological conditions. We conclude that thema nodosum. Only one patient was admitted to a hospital a reasonably heavy workload of referrals was received and that bed. Six out of 15 cellulitis patients were also diagnosed and patients were seen in a timely manner. Poor written referrals treated for an underlying dermatological condition identified with no contact number or bleep number provided meant that as the primary cause of cellulitis. Intravenous antibiotics were it was difficult to consult with the referring physician in order administered by outpatient attendance or in the patients’ own to help prioritize referrals or feed back. We therefore felt that homes. A total of 141 bed days were saved. A dermatology a formal proforma (as used in our local teaching hospital) cellulitis service can avoid incorrect diagnosis, unnecessary which includes boxes for a bleep number, suggested diagno- treatment and hospital admissions. The shorter pathway to sis, senior doctor to co-sign the form and an option for the diagnosis and treatment with effective treatment plans ensures patient to be reviewed as an outpatient would all potentially safe discharge into primary care. Treating underlying dermato- improve our service and reduce the number of unnecessary logical disease reduces the risk of recurrent episodes. Sustain- referrals. Our audit has highlighted that there are significant ing this service requires appropriate staffing, a designated numbers of inpatients requiring specialist dermatology opin- treatment area and good communication between secondary ion. With the potential move towards community-based and primary care. dermatology services, will hospital inpatients have to wait longer to receive specialist inpatient opinion?

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P-65 P-66 Analysis of inpatient dermatology referrals: to How well is the skin cancer Improving Outcomes optimize the educational needs of trainee doctors Guidance being followed? K. Ahmad and B.C.P. Ramsay S. Cohen and A. Ferguson Midwestern Regional Hospital, Limerick, Ireland Derbyshire Royal Infirmary, Derby, U.K. Aim: To identify the educational needs of junior/trainee doc- February 2006 saw the publication of the National Institute tors by analysis of inpatient consultation referrals to the Der- for Health and Clinical Excellence (NICE) guidance on cancer matology Department. Methods: In January 2007, we reviewed services, ‘Improving Outcomes for People with Skin Tumours the consultation data of 703 inpatients referred to the Derma- including Melanoma (IOG)’. This sought to standardize the tology Department between 2001 and 2006 that had been ways in which patients with skin cancer are managed. Key stored on a Filemaker Pro database. These were a randomized recommendations included that all health professionals who subset and represented approximately 50% of all inpatient knowingly treat skin cancer should be members of a skin can- consultations in the Dermatology Department in that time per- cer multidisciplinary team (MDT). Patients with low-risk basal iod. Results: 703 consultation records were identified. The ages cell carcinoma (BCC) could be treated in the community by of patients ranged from a 33-week premature baby with can- general practitioners with specialist interest (GPwSI); those didal septicaemia to a 92-year-old woman with facial contact with high-risk BCC, squamous cell carcinoma (SCC), malig- dermatitis. Patients were referred from all wards in the hospi- nant melanoma (MM) or where diagnosis was uncertain tal across all the specialties. One hundred and seventy-one should be referred. The document specified that this practice (25%) referrals were paediatric and 160 (22%) patients were should be subject to audit. Accordingly, we performed a retro- 65 years of age or older; 371 patients (52%) were admitted spective audit of all skin cancer specimens taken in the com- because of acute skin failure and this was the primary reason munity and sent for histological analysis at our hospital from for their admission. In 391 cases (55%) the consultant July 2007 until the year end. Criteria were as follows, with dermatologist’s diagnosis was different to the inpatient referral standard in parentheses: 1. Differential diagnosis or descrip- diagnosis on the consultation referral form. There were a total tion of lesion supplied (90%); 2. Site of operation stated of 113 different dermatological diagnoses in the group. One (100%); 3. Management in primary care in line with the IOG hundred and fifty-nine consultations (22%) were for skin (100%); 4. Complete excision rate where relevant (90%). Data infections; 89 patients (12%) had atopic dermatitis and 60 were extracted from histology request forms and reports. The (8%) had psoriasis; 57 (8%) had clear or suspected drug cause pathology department received 41 specimens from 22 surger- for their rash and referral. A total of 388 patients (55%) ies. None of the operations were performed by a GPwSI, or required one consultation visit but 45% required two or more other member of the multidisciplinary team (MDT). One hist- review visits from the dermatology team. This figure was ology request form was missing, but most parameters were higher in the paediatric patients referred: 76% of paediatric available from the histology report. Of the remaining 40, 30 referrals (135 of 177 patients) were admitted with acute skin (75%) were accompanied by a description of the lesion or disease as the primary or main reason for their admission. differential diagnosis; for one, no clinical information was Of these 82% required two or more visits during that admis- provided and the others were described as ‘lesion’, with no sion. Two hundred and sixty patients (36%) required follow additional information. For 39 specimens (95%), the site of up in dermatology outpatients after their admission. Discussion: the biopsy was stated. Fifteen of the lesions biopsied were sus- The dermatological conditions seen ranged from minor to pected skin cancer (including one MM and two SCCs), 12 life-threatening dermatoses and acute skin failure. Twenty per were thought to be benign and for 14, there was insufficient cent of referrals were for patients with the two commonest information to tell; compliance with the IOG was at best 63%. inflammatory dermatoses (atopic dermatitis and psoriasis) Of the 26 cancers deliberately excised, 18 (69%) were done which reflects poorly on facilities available for their care in so completely. A good deal of skin cancer surgery is still the community and hospital outpatient setting. In more than undertaken in primary care around our city, despite the half of the referrals the dermatologist’s diagnosis was different absence of GPwSI. The problems with misdiagnosis and to that of the admitting team. This has important implications incomplete excision tend to support the guidance set out by on clinical outcome as appropriate treatment and short length NICE. GPs and primary care trusts need to be aware of the of stay would be dependent on accurate and timely diagnosis. IOG and active in its implementation. Conclusions: This study emphasizes the need for junior dermatol- ogy trainees to undertake extra training in both the dermato- logical conditions of paediatric patients and those with older skin as they form a significant component of the inpatient referrals to dermatology.

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P-67 P-68 Are NICE skin cancer guidelines being followed Patients with melanoma are having inappropriate in primary care? A review of current practice in an surgical interventions in primary care: an audit inner city setting A.-M. Skellett, E. Tan and J. Garioch R. Healy, D. Rahman, K. Gibbon, A. Sahota and Norfolk and Norwich University Hospital NHS Trust, Norwich, U.K. A.P. Bewley Patients with melanoma should not have biopsies or excisions Whipps Cross University Hospital, London, U.K. carried out in primary care (Roberts DLL, Anstey AV, Barlow Skin cancers are extremely common with approximately RJ et al. U.K. guidelines for the management of cutaneous mel- 80000 new cases registered each year in the U.K. (UK Malig- anoma. Br J Dermatol 2002; 146: 7–17; National Institute for nant Melanoma Incidence Statistics, Cancer Research UK 2007. Health and Clinical Excellence (NICE). Referral guidelines for Available from: http://info.cancerresearchuk.org [last accessed suspected cancer. Available from: www.nice.org.uk/CG027 15 February 2008]). This is thought to be a significant under- [last accessed 16 February 2008]). Audit data have estimated estimate of the true incidence, as many cases of nonmelanoma that 1.4–13% of melanomas are inappropriately biopsied in skin cancer (NMSC) are not registered. There are large num- primary care (NICE). The aim of this audit was to identify bers of patients requiring treatment of skin malignancies, whether patients with melanoma in our region are having in- which represents a considerable burden for health services. In appropriate surgical interventions in primary care before being February 2006 the National Institute for Health and Clinical referred to secondary care. Patients with a diagnosis of mela- Excellence (NICE) issued guidelines on the organization of noma were identified from our histopathology database skin cancer services. Its key recommendations included: pre- between 1 January and 31 July 2006 and for the same period cancerous lesions and low-risk basal cell carcinomas (BCC) in 2007. From the histopathology reports, we were able to can be treated in primary care by appropriately trained indi- identify patients who had had biopsies or incompletely viduals (usually a GP with a special interest); all other skin excised melanoma excisions performed by GPs. We excluded malignancies should be treated, at least initially in primary biopsies taken from acral sites, lentigo maligna and locally care; all doctors treating skin cancers should be part of the recurrent or metastatic melanoma. Ninety-five melanomas local multidisciplinary team (MDT) and participate in audit. were identified from January to July 2006 and 80 over the Our aim was to determine how many skin cancers were being same time period in 2007. Four out of 95 had been incom- diagnosed and treated in primary care in an inner city London pletely excised or biopsied (2 biopsies, 1 curettage and 1 location over a 12-month period and to assess whether the incomplete excision) in 2006 in primary care, and 10 out of NICE recommendations were being followed. We identified 80 in 2007 (5 incomplete excisions, 3 curettages and 2 biop- all patients on our histopathology database who had a skin sies); six of these 10 lesions were suspicious of melanoma at biopsy in primary care and a diagnosis of skin malignancy the time of operation as suggested by the clinical information documented over a 12-month period from 1 April 2006 to on these patients’ histology forms, e.g. ‘ulcerating, rapidly 31 March 2007. A total of 151 patients including 11 malig- enlarging lump from pre-existing mole’. Our figures show nant melanomas (MM), 18 squamous cell carcinomas (SCC) that the percentage of lesions biopsied or incompletely excised and 122 BCC were identified, classified according to subtype in primary care has more than trebled from 2006 to 2007 and the excision margins were documented. Of the 11 MM (from 4% to 12Æ5% of the total number of melanomas patients 10 were referred to secondary care, but one patient referred to our multidisciplinary team). The NICE Improving had been missed as the histology report had not been read by Outcomes Guidance states that the number of patients with the GP. As a result of this audit the patient was informed and melanoma having biopsies in primary care should reduce fol- referred to secondary care for a wide local excision and senti- lowing implementation of the guidance, but in our region, nel lymph node biopsy. Eleven out of 18 (61%) of SCC were the reverse has been found to be true. Inappropriate surgical incompletely excised. Only 47% of BCC were completely interventions in primary care are leading to delays in these excised; 61% of incompletely excised BCC were not referred patients being referred to secondary care for their definitive to secondary care for re-excision. Our findings highlight that treatment. We predict that if this trend continues then patients large numbers of skin cancers are being treated inappropriately will present later to secondary care with thicker, poorer prog- in primary care and there are high rates of incomplete surgical nosis tumours or with metastases. A prospective study is being excision. NICE skin cancer guidelines are frequently not being planned to examine this hypothesis. adhered to, which appears to be exposing many patients to unnecessary risk.

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 56 Bristol Cup Posters

P-69 P-70 Skin cancer biopsies in primary care: neither BAD Understanding delay: a Grounded Theory nor NICE examination of the prediagnostic journey M. Mehra, R. Stitson, J. Natkunarajah, S. George, of individuals with malignant melanoma C.C. Harland and A. Abdul-Wahab I. Nyawata, A. Topping and A. Wright Epsom and St Helier NHS Trust, Carshalton, U.K. University of Bradford, Bradford, U.K. Skin cancer management in primary care has been suboptimal. The incidence of melanoma is rising at a rate faster than that of Clinical audits from NHS Trusts in England have shown that any other type of cancer. Health promotion campaigns advo- the proportion of malignant skin tumours biopsied in primary cating harm minimization by covering up, using skin protec- care ranges from 1Æ2% to 17%, comprising up to 13% of tion and adopting avoidance behaviour have been the mainstay malignant melanomas, 10% of squamous cell carcinomas of prevention. Melanoma is a curable malignancy and survival (SCCs) and 8Æ8% of basal cell carcinomas (BCCs). In our own prospects are associated with early detection and thickness of audit we demonstrated a worrying failure by general practi- tumour at time of diagnosis. When detected and treated early, tioners (GPs) to manage skin cancer cases in the community. melanomas usually have a highly favourable prognosis. Never- Of 896 skin biopsies sent to our histopathology department theless a substantial number of individuals present for treat- during a 6-month period, 31 were BCCs and 5 were SCCs. ment with extensive invasion of disease on diagnosis. There is Forty further specimens were inadequate for definitive histo- limited evidence examining why presentation delay occurs – logical interpretation. There was also one malignant melanoma something this study sought to rectify. The aim of the study and one porocarcinoma. Only 25% had clear margins, but was to explore the prediagnostic experiences of individuals none were considered adequately excised. Only 29% of patients diagnosed with malignant melanoma, mapping the journey who had a BCC removed in primary care were referred for from problem awareness to diagnosis in order to illuminate follow-up (mean interval 81 days). For those with SCCs, 40% the influences on delay. Qualitative conversational interviews were referred (mean interval 25 days). Subsequently, in 2006, with a purposive sample of individuals (n = 42), diagnosed The National Institute for Health and Clinical Excellence with malignant melanoma were undertaken, complemented by (NICE) published guidelines for the management of skin can- a theoretical sample of individuals with superficial tumours cer. It was recommended that patients with suspected skin (n = 6) who were described as nondelayers at diagnosis. Inter- cancer (especially SCCs and melanoma) should be referred to views were audiotaped, transcribed and analysed using the secondary care for specialist management. In 2007 we Grounded Theory approach described by Charmaz (Charmaz K. re-audited primary care biopsies received over a 2-month per- Constructing Grounded Theory: a Practical Guide through Qualitative Analysis. iod against the NICE guidelines. In total we received 373 skin London: SAGE Publications, 2006). Constant comparison specimens from GPs. Of these 16 were skin cancers, with 5 underpinned data collection and analysis. NVIVO 7 was used SCCs and 11 BCCs. In 6 out of 16 cases skin cancer was sus- to support analysis. Six themes that best conveyed the experi- pected at the time of the biopsy and only 2 of these were ences of the informants emerged from the study. These are taken by a trained GPSI. In the other 10 cases, no clinical referred to as ‘the portrait of a delayer’ and provide a thick details were given or the suspected diagnosis was incorrect. description of the characteristics of individuals who delay. The However, a standardized prompt by the histopathologists to themes – (i) misinterprets symptoms, (ii) fails to make the refer to secondary care per NICE guidelines appears to have connection, (iii) is a man, (iv) is a busy person, (v) is not resulted in rapid 2-week referrals. These results suggest that comfortable visiting the doctor and (vi) previous benign diag- the number of skin biopsies of potentially malignant skin nosis – comprehensively describe the presentation journey and lesions being carried out in general practice remains signifi- the complexities of decision-making. Delayed presentation is cant despite recent national guidelines. GPs are still biopsying one of the major challenges in cancer care. This paper presents suspected malignancies and skin lesions for which they are an interpretation of the experiences of individuals who present unsure of the diagnosis. Nevertheless skin cancer multidiscip- late with malignant melanoma and offers an understanding to linary teams in secondary care are empowered to advise GPs; inform effective health education/promotion. for example, the histopathology report itself can recommend referral. This pilot audit will be rolled out across the skin can- cer network. Our aim is to present our findings to our neigh- bouring Primary Care Trusts (PCTs) and to the Skin Cancer Tumour Working Group (TWG). Thus, not only will GPs be P-71 Experience of biological therapy in a teaching made aware of the guidelines, but also GPs identified as hospital dermatology department, with attention performing skin cancer biopsies could be targeted by the to concomitant medications, other diagnoses and PCT/TWG for retraining. side-effects S. Rajpopat, A. Waite, M. Rustin and S.R. McBride The Royal Free Hospital, London, U.K. Biological therapies are increasingly used in the treatment of psoriasis and other skin diseases. Concern remains regarding

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 57 long-term safety. The use of biological agents in a clinical set- treatment and symptomatic patients, as indication for the ting differs from a trial situation in which much of the avail- urgent referral. Seventeen per cent of cases were paediatric, able safety data are obtained. This study investigates the use of and 4% were pregnant women. A proportion of GP letters biological agents in a dermatology teaching hospital depart- provided no reason for urgency. The dermatology team felt ment with attention to the use of concomitant medications, 13% of patients seen would have been more appropriate for a other medical diagnoses and side-effects. A retrospective notes routine appointment. These were patients with stable, chronic review of 37 patients, treated with one or more biological problems such as acne vulgaris, psoriasis and viral warts. A agents was performed. Sixty-five courses of treatment were further 6% presented with possible skin cancers, which should given: etanercept n = 35; infliximab n = 20, efalizumab n = have been referred to the Skin Cancer Screening clinic via the 4, adalimumab n = 3 and rituximab n = 3. Sixteen of these usual 2-week rule pathway. Eleven of 52 inappropriate walk- were in a trial setting. Diagnoses included: psoriasis n = 31, in referrals were received from a single GP. The majority of pemphigus n = 3, atopic eczema n = 1, hidradenitis suppurati- patients did present to the Dermatology Department within a va n = 1 and pyoderma gangrenosum n = 1. Outside of trials few days of consulting their GP. Eleven per cent of patients 43 courses were given for psoriasis; 84% had concomitant attended more than a week from the date of the GP referral treatment: methotrexate (MTX) n = 14, fumaric acid esters letter, with 6 patients attending more than a month later. Two (FAE) n = 5, ciclosporin n = 4, hydroxyurea n = 1, MTX + patients were admitted from the walk-in clinic, and 43% were FAE n = 1. Significant other diagnoses were previous meta- reviewed again within 1 month, suggesting a significant skin static thyroid carcinoma, alcoholic liver disease and squamous disorder; 35% were discharged back to the care of the GP cell carcinoma (SCC) – none worsened with treatment. Side- from the walk-in clinic. Twenty-three per cent of patients effects with etanercept included night sweats and haemoptysis, failed to attend their follow-up appointments, which may generalized dysaesthesia, rash, angio-oedema, marrow failure reflect the rapid resolution of an acute rash. It was anticipated and lung infiltration secondary to vitamin B12 deficiency and that a large number of biopsies may be generated from SCC. Side-effects of infliximab included infusion reactions, patients with acute, often undiagnosable rashes. Forty-two drug-induced hepatitis and erythrodermic flare of psoriasis. patients were booked in for a biopsy, of which six did not Efalizumab caused a severe flare of arthritis in 2 of 4 treated. attend. In 10 cases the histology was not diagnostically help- Our limited experience of biological therapy is encouraging in ful. A survey of patients and local general practitioners has terms of safety and efficacy although the majority of psoriatic shown the service to be well received. The viability of derma- patients require concomitant immunosuppressive therapy. Fur- tology departments has been placed under threat by NHS poli- ther awareness of the use of biologicals in the clinical rather cies restricting access to secondary care. This local initiative than trial setting is required. was introduced to facilitate access to a dermatology opinion for more severe cases.

P-72 Evaluation of the dermatology walk-in clinic at a P-73 London teaching hospital Five years’ experience of a dermatology e-mail L. Simpson, N. Desai, V. Pinder and R.A. Marsden advice service St George’s Hospital NHS Trust, London, U.K. S. Walsh and D. Kemmett Dermatology is under threat, due to restrictions on secondary Department of Dermatology, Royal Infirmary of Edinburgh, Edinburgh, U.K. referrals. To redress this issue, an emergency walk-in clinic With increasing interest in the concept of telemedicine in was introduced by our department. Walk-in patients are seen dermatology and ever-lengthening waiting lists for clinics, the between 10.00 and 11.00 a.m. on weekdays without prior need for a facility by which general practitioners can access appointment, as long as they present a GP letter marked as expertise remotely is rising (Finch TL, Mair FS, May CR. Tele- urgent. To the best of our knowledge, this is the only service dermatology in the UK: lessons in service innovation. Br J Der- of its kind in the U.K. In our first 6 months, March to August matol 2007; 156: 521–7). We report our experiences of an 2007, a total of 272 patients were seen in the walk-in clinic, e-mail advice service operating in our Trust since 2003. Over with an upward trend in attendances. Eighty-six per cent of this period, over 2000 enquiries have been dealt with. We referrals came from the six postcode areas immediately sur- evaluated the nature of the enquiries that are made of the ser- rounding the hospital. There was initial concern about unpre- vice by examining in detail e-mail enquiries received over a dictable numbers of extra patients attending the department. 4-month period. We compared these results with an evalu- Daily walk-in numbers remained manageable over the first 6 ation of the service carried out in 2004 in order to ascertain months. Three patients or fewer were seen on 81% of days, how the service has evolved. We also evaluated response to with a maximum of seven patients seen on two occasions. GP the service by means of a questionnaire distributed to GPs letters most commonly stated an acute, widespread or deterio- who had availed themselves of the service. The mean number rating rash, uncertainty of diagnosis, failure to respond to of referrals per month in 2007 was 60 (compared to a mean

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 58 Bristol Cup Posters of 28/month in 2004), and the majority of these (78%) were Only 8 (22%) patients were given advice about soap substi- replied to within 3 days. The median time to reply was in fact tutes. Psychosexual dysfunction was only discussed with one < 24 h. We examined a sample of the GP queries received patient. Twenty-five (69%) patients were followed up within over a 4-month period (n = 253) from July–October 2007. 3 months in accordance with guidelines. Information regard- The most common enquiry was regarding an unidentified rash ing malignancy risk, patient information leaflets and patient requiring diagnosis and/or management advice (24%). Que- support groups was patchy or poorly documented. None of ries regarding management of eczema or psoriasis accounted the nine discharged patients was advised to see their GP for for 21%. Fourteen per cent of enquiries were regarding yearly review. In the re-audit, 15 patients aged between 4 and lesions such as suspected skin cancers. Smaller numbers of 86 years (mean age 55 years) were identified. All had genital enquiries were received about conditions such as acne/rosa- LS with two also having extragenital disease. All were seen by cea, alopecia, pigmentary disorders, and hirsut- a vulval dermatologist. All patients were initially prescribed a ism. Of 132 questionnaires distributed to general practitioners very potent corticosteroid and given advice about soap substi- who had used the service in the preceding 4 months, 63 com- tutes. Frequency and time period of corticosteroid use was pleted questionnaires were returned. Most respondents (58%) documented in 13 (86%) patients. Psychosexual dysfunction had used the service on 1–4 occasions in the past, with a fur- was discussed with all 8 (53%) sexually active patients. All ther 25% having used the service on more than 4 occasions. patients were offered follow-up. Twelve were seen within 3 Satisfaction levels with the service among users were high, months and three who had longstanding disease within 6 with most aspects being rated as ‘excellent’. We asked the GPs months. Malignancy risk was documented in one case. Patient to identify what they perceived as the benefits of the service. support groups were mentioned in three cases. Patient infor- Eighty-five per cent felt that it was of educational value and mation leaflets were documented as given in six cases. No over 90% were of the opinion that the advice had helped with patient was discharged. Risk of loss of architecture and func- the management of the patient. Fifty-eight per cent of respon- tion with inadequate corticosteroid use was not documented dents stated that the e-mail advice had avoided the need for in either audit. Compared with our previous audit, all patients formal referral of that patient to clinic, with a further 16% now receive appropriate first-line treatment and follow-up. stating that the advice had also prevented the referral of a dif- Documentation of discussion of psychosexual dysfunction has ferent patient. Over the 5-year period of its existence, the increased significantly. Documentation regarding patient infor- e-mail advice service offered by our department has evolved mation and support groups is still inadequate. However, in and changed. The volume of enquires has increased, without practice in the vulval clinic, all patients are given an informa- any diminution in levels of user satisfaction, which remains tion leaflet with information about support groups and high. Almost 60% of responses averted the need for formal potential complications including malignancy. This re-audit patient referral, reducing the burden on clinics. E-mail advice highlights the need to change the standard clinic proforma to has become a valuable and highly regarded part of the derma- include documentation of this information according to the tology service offered in our region. BAD guidelines.

P-74 Lichen sclerosus revisited. Further evidence for a P-75 specialist vulval clinic Recent trends in dermatology referrals in England C. Soon, M. Mehmi and S.S. Velangi A.J.G. McDonagh City Hospital, Birmingham, U.K. Royal Hallamshire Hospital, Sheffield, U.K. A retrospective audit of patients with a histological diagnosis U.K. Department of Health data on dermatology referrals in of lichen sclerosus (LS) during 1996–2006 was conducted in England reveal wide apparent variation in new patient referral our department in 2006. The audit aimed to assess variations rates between PCTs ranging from under 10 per 1000 per in practice against the British Association of Dermatologists annum in the lowest referring areas to 34/1000 in the highest (BAD) guidelines prior to the introduction of a specialist vul- for 2005–6. This study presents an examination of temporal val clinic. We present the results of a re-audit conducted in trends in dermatology and all speciality rates and their patterns 2007, 1 year after a specialist vulval clinic had been set up. In of geographical variation with the objective of assessing possi- our previous audit of 36 patients, 15 (42%) were seen by a ble sociodemographic determinants of low or high referral dermatologist, 20 (56%) by a gynaecologist and 5 (14%) rates. Unlike malignant melanoma and nonmelanoma skin were seen by both. There was variation in treatment regimens cancer which show strong association of high incidence with 20 (56%) prescribed a very potent corticosteroid, 6 with residence in more affluent areas (P < 0Æ001), the (17%) a potent corticosteroid, and 5 (14%) asymptomatic overall pattern of dermatology referral rates by PCT patients were not given anything. Frequency and time period shows only weak association with indices of deprivation or of corticosteroid use were documented in 18 (50%) patients. affluence. However, significant geographical variation in

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 59 referral rates can be demonstrated when the data are grouped without a hospital outpatient appointment first, and preferred according to the Office of National Statistics Classification of this route of referral. Only one patient opted to be seen in the towns. Using this classification, PCTs in the ‘Regional Centres’, hospital outpatient clinic prior to surgery. From the surgeon’s and ‘London Cosmopolitan’ groupings have the highest refer- point of view the teledermatology images allowed appropriate ral rates overall while the lowest referral rates are associated planning of each surgical procedure with adequate timing in with Prospering Smaller Towns and New & Growing Towns. all cases. Teledermatology has not been widely accepted into In the decade since 1998, dermatology referrals to secondary clinical practice in the U.K., and has many limitations for the care (about 7–8% of new NHS outpatient referrals) have diagnosis and treatment of patients with rashes and inflamma- shown a rising trend broadly in line with all speciality tory skin lesions. However, teledermatology is extremely new outpatient referral rates with an overall increase in the effective for imaging single well-defined lesions such as basal region of 20% that became maximal in 2004–5 followed by a cell carcinomas, and allows appropriate surgical planning in significant fall in the number of referrals to secondary care the majority of cases. Our experience with teledermatology in 2005–6 and 2006–7. This fall in dermatology referrals over the last 6 years has identified a niche for the use of tele- was proportionally larger than the concomitant reduction in dermatology for triaging patients with basal cell carcinomas all-specialty rates. The new trend probably reflects changes in directly onto skin surgery lists. Many dermatologists will still service provision but at present there is a lack of comparative consider it desirable to see patients with basal cell carcinomas published data on provision in reorganized services operating in the outpatient clinic first to plan their operation, followed outwith the traditional provider framework, limiting interpre- by a separate appointment for surgery at a later date. How- tation of the effects of this complex restructuring in NHS care. ever, patients with basal cell carcinomas are often elderly with To maintain the capacity adequately to monitor the provision associated comorbidity that can make travel to the hospital dif- of dermatology services, the BAD may need to undertake ficult, and therefore modern routes of referral that avoid future data collection on members’ workload in a regular and unnecessary travel are desirable. This study demonstrates that detailed process. the combination of clear images and good written communi- cation can save many elderly patients a hospital visit, and is associated with very high levels of patient satisfaction. In our study patients were saved a round trip to the hospital of up to 50 miles, with significant benefits for patient convenience and cost, as well as the environment. P-76 Simple excision of basal cell carcinomas: patients prefer to be booked in for surgery via teledermatology referral rather than via the outpatient clinic P-77 C.R. Charman Systematic review of the safety of antihistamine use Department of Dermatology, Royal Devon and Exeter Hospital, Exeter, Devon, in pregnancy U.K. C-C. Chi, C-W. Lee,* G. Kirtschig and The aim of this ongoing study is to assess the use of teleder- F.T. Wojnarowska matology for triage of patients with basal cell carcinomas Nuffield Department of Clinical Medicine, University of Oxford, Department directly onto skin surgery lists for excision and primary clo- of Dermatology, Churchill Hospital, Oxford, U.K.; *Outcomes Research & sure. Patients suspected to have a basal cell carcinoma by their Evidence Based Medicine, Pfizer Ltd, Surrey, U.K. and Department of general practitioner were photographed in the community. Dermatology, VUMC, Amsterdam, the Netherlands Photos and a scanned referral letter were received by the con- We conducted a systematic review of the safety of antihista- sultant dermatologist in the local hospital via a secure e-mail- mine use in pregnancy, and searched 15 electronic databases based teledermatology system. Patients were triaged directly as well as bibliographies. Relevant randomized controlled trials onto a skin surgery list and sent surgery information in the (RCTs), prospective cohort studies with at least 100 exposed post, with the option of arranging a hospital outpatient subjects, and case–control studies with at least 20 cases were appointment prior to their surgery if required. Thirty consecu- included. Studies limited to antihistamines that are not used tive patients were sent a postal questionnaire after their opera- for skin conditions, e.g. doxylamine and meclizine, were tion to assess patient satisfaction with the service. Thirty excluded. One RCT, 15 cohort and 7 case–control studies questionnaires were returned. One hundred per cent of were eligible for this review. Ten studies assessed long-acting patients said they received enough information about their antihistamines, with two examining terfenadine, which has operation in the post before their surgical appointment. been withdrawn from the market. Meta-analysis was inappro- Ninety-three per cent of patients (28 of 30) were very satis- priate and not performed. Most studies did not show an asso- fied and 7% were quite satisfied with the care they received ciation between antihistamine use in early pregnancy and during their operation. Twenty-nine of 30 patients (97%) congenital malformation. However, brompheniramine intake were happy to have been booked straight in for surgery in early pregnancy was associated with congenital malformation

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 60 Bristol Cup Posters

[relative risk (RR) 2Æ34, P < 0Æ05] (Heinonen 1977). One Intensity of itching and wealing was assessed with visual ana- case–control study reported a significantly increased risk of logue scales (VAS) and the wealing response was evaluated by oral cleft in women who had taken diphenhydramine in the testing with a dermographometer at pressures of 20, 36 and first trimester [odds ratio (OR) 3.36, 95% confidence interval 60 g mm–2 on the upper back. NB-UVB phototherapy was (CI) 1Æ27–9Æ41, calculated from reported data] (Saxe´n 1974). given for 6 weeks three times weekly starting at 50% of mini- Hypospadias had been associated with loratadine exposure in mal erythema dose with 20% to 0% increments as tolerated. a Swedish Medical Birth Register study (RR 2Æ39, 95% CI Fexofenadine 180 mg daily was taken during the run-in per- 1Æ43–3Æ38) (Ka¨lle´n 2001), but this relationship was not con- iod and subsequently throughout the study and follow-up as firmed by continued surveillance of the same register (RR required. Patients were followed for 3 months with regular 0Æ47, 95% CI 0Æ06–1Æ68) (Ka¨lle´n 2006) and other case–con- assessments every 6 weeks after completion of phototherapy. trol studies [OR 0Æ96, 95% CI 0Æ62–2.68 (Werler 2004); OR All patients showed an improvement in itching at the end of 1Æ4, 95% CI 0.0–10Æ5; and OR 0Æ9, 95% CI 0Æ1–6.9) (Peder- NB-UVB treatment [mean (SD) reduction 52Æ3% (31Æ6%)]. sen 2006)]. One case–control study showed a protective effect Subjective assessment of wealing revealed a significant of antihistamine (OR 0Æ30, 95% CI 0Æ08–0Æ87, calculated from improvement in all but two patients [mean (SD) reduction reported data) (Nelson 1971). The RCT and eight cohort stud- 71% (54%)]. There was small and statistically significant ies found no differences in the risk for fetal loss between the improvement in cumulative dermographometer-induced weal exposed group and controls. Another cohort study showed a widths at the end of phototherapy (P = 0Æ04). A time trend lower risk of stillbirth in the exposed group (RR 0Æ60, 95% CI for the relapse of symptoms within 12 and 18 weeks after 0Æ40–0Æ89, calculated from reported data) (RCGP 1975). completing phototherapy was significant for both VAS scores There were five cohort studies measuring fetal growth and but not for dermographometer-induced wealing. NB-UVB gestational age. Only one study found a marginally significant phototherapy is an effective second-line treatment for patients lower birth weight (P = 0Æ04) but no increased risk of low with severe symptomatic dermographism responding poorly birth weight and small for gestational age in the terfenadine- to fexofenadine. This therapy can lead to subjective relief of exposed newborns (Loebstein 1999). Four, three and one pruritus and wealing and objective reduction of wealing. NB- cohort studies examined the mode of delivery, neonatal dis- UVB phototherapy may restore symptom control with antihis- tress and maternal pre-eclampsia, respectively; no significant tamines in some patients. differences were noted. Zierler (1986) suggested an associa- tion between antihistamine use in the last 2 weeks of preg- nancy and retinopathy of prematurity (RR 2Æ1, CI not reported). These data suggest a lack of association between maternal use of antihistamine and adverse perinatal outcomes P-79 including fetal loss, fetal growth and mode of delivery. To compare the response of mucosal lesions in However, the overall quality of evidence is unsatisfactory, and pemphigus vulgaris to pulse vs. oral conventional relevant data of long-acting antihistamines are insufficient. steroids R. Jain IMA Hospital, Chandigarh, India The aim of the study was to compare the response of mucosal lesions in pemphigus vulgaris (PV) to pulse vs. conventional oral steroids. Patients with mucosal PV were divided P-78 Methods Narrow-band ultraviolet B phototherapy is beneficial into two groups. Group 1 consisted of patients with pure in antihistamine-resistant symptomatic mucosal pemphigus who were treated with daily oral conven- dermographism tional steroids. Group 2 consisted of patients with mucosal as E. Borzova, A. Rutherford, G. Konstantinou, well as cutaneous lesions who were treated primarily with K. Leslie* and C. Grattan dexamethasone–cyclophosphamide pulse (DCP) therapy. Oral Norfolk & Norwich University Hospital, Norwich, U.K. and *University of prednisolone was added only if DCP alone failed to control California, San Francisco, U.S.A. the disease. The severity of the mucosal lesions was graded as: Symptomatic dermographism is the commonest of the physi- grade I, < 2 oral mucosal areas and no difficulty in eating; cal urticarias. It can be severe and poorly controlled with H1 grade II, > 2 oral mucosal areas and difficulty in taking solids; antihistamines in some patients. Photochemotherapy (PUVA) grade III, > 2 mucosal areas and severe eating difficulty. Results may help the itch of dermographism but narrow-band ultravi- Group 1 consisted of 34 patients with a mean duration of olet B (NB-UVB) therapy has not been studied for this condi- mucosal lesions of 7Æ3 months. The mean dose of oral pred- tion before. The aim of the study was to examine the clinical nisolone required to treat grade III mucosal lesions was 28Æ6 efficacy of NB-UVB therapy for itch and wealing in symptom- mg daily for a mean duration of 33Æ6 weeks, and for grade II atic dermographism and to assess the duration of the effect the dose was 20 mg daily for 21Æ8 weeks. In group 2 (68 over 3 months of follow-up. Eight patients (six female) were patients with mucosal lesions of a mean duration of 4Æ7 enrolled into an open, uncontrolled prospective study. months), to treat grade III lesions, in addition to a mean of

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14Æ1 DCP, a mean dose of 23Æ4 mg daily for a mean of 28 acquired with efalizumab (Raptiva) (CLEAR) trial in patients weeks was required. Grade II lesions in group 2 required, in with moderate-to-severe plaque psoriasis: results from a phase addition to a mean of 10.3 DCPs, a mean dose of 15 mg of III international randomized, placebo-controlled trial. Br J prednisolone for a mean of 21Æ7 weeks. Grade I lesions in Dermatol 2006; 155: 170–81; Woolacott N, Hawkins N, Mason group 2 responded to DCP alone. DCP alone failed to improve A et al. Etanercept and efalizumab for the treatment of psoria- pure mucosal lesions, tried separately, and these patients were sis: a systematic review. Health Technol Assess 2006; 10: 1–233, treated with oral prednisolone. More remissions occurred in i–iv), the arthropathy was unusual. This case illustrates the group 1 and they were longer lasting. The same relapse rate importance of being aware that severe and disabling psoriatic of 40% occurred in both groups. In conclusion, mucosal arthritis can be unmasked by the use of efalizumab and should lesions of moderate to severe intensity respond only to daily not be dismissed as a mere side-effect of the drug. oral prednisolone with or without DCP. DCP alone can be used to treat grade I mucosal lesions.

P-81 A case of adalimumab-induced lupus erythematosus F. Al-Niaimi and D.M. Tillman P-80 Western General Infirmary, Glasgow, U.K. Efalizumab unmasks psoriatic arthritis Drug-induced lupus erythematosus is a syndrome with symp- M. Sharma and R. Murphy toms, signs and laboratory findings similar to idiopathic sys- Queen’s Medical Centre, Nottingham, U.K. temic lupus erythematosus. Central nervous system and renal A 43-year-old gentleman with a history of severe psoriasis for involvement are rare. More than 80 drugs have been associ- over 15 years attended our dermatology department. After trial ated with this condition with sulfadiazine being the first with intensive topical treatment, he had phototherapy with reported in 1945. The condition is usually reversible with the acitretin which was ineffective. Over the next decade he had withdrawal of the drug (Vasoo S. Drug-induced lupus. An multiple systemic therapies including ciclosporin which was update. Lupus 2006; 15: 1296–9). A 43-year-old woman who discontinued due to renal impairment and hypertension. He was being treated with adalimumab for rheumatoid arthritis was then tried on methotrexate and fumaric acid esters which developed erythematous patches with telangiectasia on the caused deranged liver function and his skin did not improve. face, shoulders and back several weeks after starting ada- His psoriasis remained severe with a PASI score of 15Æ4 and limumab. Biopsy of the lesions was consistent with lupus ery- DLQI of 14, and biologic agents were considered. He was thematosus. Her connective tissue screen, previously commenced on etanercept 25 mg twice weekly. He did rela- persistently negative, showed rheumatoid factor 1260 IU mL–1, tively well, but failed to achieve PASI 50 or 75 at the end of antinuclear antibody (Ab) (titre 1/640) and a weak positive week 12 and was subsequently switched to efalizumab at 1 mitochondrial Ab titre of 1/50. She was negative for dsDNA mg kg–1 subcutaneously. However, within 5 weeks of com- Ab. Her urinalysis was negative for blood and protein. Full mencement of the drug, he noted marked morning stiffness blood count and electrolytes were normal. She also had symp- with fleeting joint pains in his jaw, chest, shoulders, elbows, toms of myalgia and increased photosensitivity, both of which knees and ankles. He had some flu-like symptoms and the were new symptoms. Adalimumab was withdrawn by the joint pains kept him awake at night. On examination, there rheumatologists and substituted by azathioprine. High-factor was tenderness in his temporomandibular, costochondral and sunscreens were applied but the patient could not tolerate top- ankle joints but no obvious associated joint swelling. X-rays ical steroids. Although currently unlicensed in cutaneous revealed classical bilateral sacroiliitis and changes in keeping lupus, topical tacrolimus (Protopic, Astellas, Staines, U.K.) was with chronic psoriatic arthritis. He went on to develop signifi- used with success in our patient. Her symptoms of myalgia cant dactylitis. Prior to starting efalizumab, he did not have and photosensitivity improved with an overall improvement any symptoms suggesting psoriatic arthopathy. Our patient’s in her cutaneous condition. Positive antinuclear antibodies musculoskeletal symptoms and signs were probably masked with or without cutaneous signs have been reported with the by the anti-inflammatory and systemic immunosuppressive biological agents infliximab and adalimumab (Benucci M, Li therapy. Efalizumab helped improve his psoriasis but his pso- Gobbi F, Fossi F et al. Drug-induced lupus after treatment with riatic arthritis became more apparent. Although mild chills, infliximab in rheumatoid arthritis. J Clin Rheumatol 2005; 11: abdominal discomfort, headache and flu-like symptoms have 47–9). Clinicians should be alert to this relatively rare but been described as typical adverse reactions with efalizumab reversible side-effect with the increase in the use of biological (Dubertret L, Sterry W, Bos JD et al. Clinical experience therapies.

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P-82 more likely to lead to the same. This is the first report of Granulomatous septal panniculitis as part of a granulomatous septal panniculitis as part of a hypersensitivity hypersensitivity reaction to azathioprine reaction to AZA. S. Orpin, N. Bahar, P. Colloby and J. Hague Heart of England NHS Foundation Trust, Birmingham, West Midlands, U.K. Azathioprine (AZA) is the nitroimidazole derivative of 6-mer- captopurine. As well as being used to prevent the rejection of transplanted organs its immunosuppressive effects have been P-83 harnessed to treat a wide variety of other conditions including Risk of herpes zoster infection in patients with rheumatoid arthritis, inflammatory bowel disease, autoim- pemphigus on mycophenolate mofetil mune disorders and atopic eczema. We present a case of a M. Saha, M.M. Black and R.W. Groves hypersensitivity reaction to AZA associated with a cutaneous St John’s Institute of Dermatology, Kings College London, London, U.K. eruption that showed a granulomatous septal panniculitis. Our The use of mycophenolate mofetil (MMF; CellCeptÒ, Roche patient, a 52-year-old woman with a 12-year history of Pharmaceuticals, Welwyn Garden City, U.K.) in dermatology Crohn’s disease (CD), started oral AZA at a dose of 50 mg is expanding particularly in immune-mediated skin disease. twice daily. Within 2 weeks she felt systemically unwell with Dermatological indications have included psoriasis and a num- fever, nausea and myalgia and she developed a rash on the ber of immunobullous diseases including pemphigus vulgaris. trunk and limbs. At presentation 5 days after the rash appeared MMF is an ester of mycophenolic acid (MPA), the active there were numerous erythematous papules over the upper metabolite which acts by inhibiting inosine monophosphate chest anteriorly, while on the limbs there were more tumid dehydrogenase in the de novo purine synthesis pathway result- dermal lesions that clinically resembled erythema nodosum ing in impaired RNA, DNA and protein synthesis. MMF acts (EN). An incisional biopsy of a lesion on the thigh showed a specifically on lymphocytes by inhibiting proliferation of both mild lymphocytic infiltrate around the upper dermal vessels T and B cells. We have treated a total of 55 patients with but no neutrophils or vasculitis. In the subdermis there was a pemphigus with MMF over a 7-year period in our department. predominantly septal panniculitis with granuloma formation Eight patients (14%) in this group have also developed local- and some infiltration of vessels with lymphocytes but no sign ized herpes zoster infection. Fifty per cent (4 of 8) of the of vascular damage. Azathioprine was discontinued at this time patients presented late due to a self-misdiagnosis of pemphi- and within a week the symptoms and the rash had resolved. gus leading to delay in initiation of treatment. All patients There has been no deterioration in her CD, or signs of meta- were on low-dose prednisolone (5–12Æ5 mg daily) and two static CD during or since the eruption. Hypersensitivity reac- patients were also on cyclophosphamide 50 mg daily. The tions are a rare but well-established adverse response to AZA. treatment dose of MMF varied between 1 g and 3 g daily. An associated rash is often reported and this can be exanthem- Treatment duration ranged between 1 and 72 months (mean atous, urticarial, erythema multiforme-like, vasculitic or as in 24 months). Those who were seen early by a physician were our case EN-like. Despite over 50 reports of hypersensitivity treated with 7 days of aciclovir and MMF was temporarily reaction with cutaneous features to AZA there is little pub- stopped. All recovered satisfactorily. The mean lymphocyte lished about the specific histopathological features. de Fonclare count at the time of development of herpes zoster for the et al. described AZA hypersensitivity in five patients with eight patients was 0Æ9 x 109 L–1, (range 0Æ5–1Æ2 x 109 L–1) inflammatory bowel disease all displaying an EN-like eruption indicating a minimal lymphopenia on treatment with MMF. (de Fonclare AL, Khosrotehrani K, Aractingi S et al. Erythema These data suggest a significant risk of herpes zoster in pem- nodosum-like eruption as a manifestation of azathioprine phigus patients on MMF. Despite minimal lymphopenia, hypersensitivity in patients with inflammatory bowel disease. patients may still be at risk of herpetic infection perhaps Arch Dermatol 2007; 143: 744–8). Histology in three cases because of the very specific nature of the effect of MMF on T showed septal panniculitis much as we observed, but unlike lymphocytes. MPA levels may have a role in predicting side- our patient there was no evidence of granuloma formation. effects and have been of value in determining the risk of viral There have been no reports of these histological features infections in transplant patients (Smak Gregoor PJH, Van Gel- related to hypersensitivity to this medication before. Granu- der T, Van Riemsdijk-van Overbeeke IC et al. Unusual presen- loma formation is complex and appears to stem from persis- tation of herpes virus infections in renal transplant recipients tence of a proinflammatory agent in the tissues either because exposed to high mycophenolic acid plasma concentration. of resistance to metabolic breakdown or deficiencies in the Transplant Infect Dis 2003; 5: 79–83). Herpes zoster in patients immune system. In CD an excessive Th1 response with over with pemphigus can easily be mistaken for the blistering production of interleukin-12, interferon-c and tumour necro- lesions of pemphigus and a greater awareness of this potential sis factor-a gives rise to granulomata and it may be that in adverse effect should aid early recognition and treatment. such an environment other types of immune reaction are

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 63

P-84 and investigator assessment; AK 04). PD P 506 A–PDT is an Excellent cosmetic outcome after photodynamic easy-to-handle one-step procedure for therapy of isolated mild therapy of actinic keratosis with an innovative to moderate AK lesions. PD P 506 A–PDT leads to excellent 5-aminolaevulinic acid patch proves superior to cosmetic results being superior to cryosurgery. This very cryosurgery important feature further supports the excellent efficacy data A. Hauschild, E. Stockfleth,* G. Popp, F. Borrosch,à of this novel formulation. H. Bru¨ning,§ R. Dominicus,– H. Mensing,** The studies were sponsored by industry. U. Reinhold, K. Reich,àà A.C.E. Moor,§§ M. Stocker,§§ C. Ortland§§ and R.M. Szeimies–– University of Kiel, Department of Dermatology, Allergology and Venerology, Kiel, Germany; *Hauttumorcentrum Charite´, Berlin, Germany; Hofackerstr., P-85 Augsburg, Germany; àHautarztcentrum, Vechta, Germany; §Tagesklinik fu¨r An in vivo comparison of the cumulative effect on Allergie – u. Hautkrankheiten, Kiel, Germany; –Hautzentrum, Duelmen, skin hydration of two topically applied formulations, Germany; **Dermatologisches Ambulatorium, Hamburg, Germany; DB gel and aqueous cream Praxisklinik, Bonn, Germany; ààDermatologikum, Hamburg, Germany; J. Gallagher, P. Rosher, J. Walker and V. Hart* §§Photonamic GmbH & Co. KG, Wedel, Germany and ––University Dermal Laboratories, Hitchin, U.K. and *RSSL, Reading, U.K. Regensburg, Regensburg, Germany The aim of this study was to evaluate the cumulative effects Photodynamic therapy (PDT) is increasingly used for the treat- on skin moisturization of an emollient gel (DB) (RSSL Pharma, ment of actinic keratoses (AK) as it is a highly effective ther- Reading, U.K.) specially formulated for increased hydration, apy leading to excellent cosmetic results. For the simplification and to compare this with aqueous cream BP (AC). AC was of PDT, a thin self-adhesive patch was developed (PD P 506 chosen as the comparator because, although originally devised A) which contains 2 mg cm–2 5-aminolaevulinic acid as a soap substitute, its relatively low cost attracts prescriptions (5-ALA). PD P 506 A can be directly applied to mild to mod- for continuous applied use when treating eczema and dry skin erate AK lesions without preparation of these by, e.g. curet- conditions. The hydration properties of AC are therefore large- tage. We report the cosmetic results observed in two ly assumed rather than proven. The study was a single-centre, confirmatory randomized parallel-group studies which were assessor-blind bilateral comparison in 20 adults with self-per- set up to investigate efficacy, safety and cosmetic outcome of ceived dry skin. Subjects were screened on day 1 of the trial PD P 506 A–PDT in comparison to placebo–PDT (superiority and given both products, presented in identical pump dis- design, observer-blinded; study code AK 03) and standard penser containers, and asked to use them separately on their therapy, cryosurgery (noninferiority design, open; study code legs (according to a preprepared random application pattern). AK 04). The primary variable in both studies was the clinical Subjects were instructed to apply the emollients in equal clearance rate on lesion basis 12 weeks after therapy. Second- amounts three times daily (at approximately 09.00, 12.00 and ary variables included pigmentation status, patient and investi- 17.00 h) for 7 consecutive days. The use of any other topical gator assessment of cosmetic results as well as overall preparation on the legs was not permitted, although subjects satisfaction with the cosmetic outcome 12 weeks after therapy. were encouraged to bathe or shower as usual. Product effec- Four hundred and forty-nine patients (AK 03: 103 patients; tiveness was assessed by hydration measurements made using AK 04: 346 patients) with a maximum of eight mild to mod- a Multiprobe Adapter MPA5 with a CorneometerÒ CM825 erate AK study lesions located on the head were randomized probe (Courage-Khazaka Electronic, GmbH, Germany). Mea- in a total of 26 study centres. A total of 2537 study lesions surements were performed in triplicate on matched skin sites (AK 03: 587; AK 04: 1950) were treated. Twelve weeks after marked on both legs. On days 1, 3 and 5, measurements were treatment, PD P 506 A–PDT was superior to placebo–PDT and made in the morning immediately prior to the first application cryosurgery with respect to treatment efficacy. All treatments of the day. On days 1, 3 and 5 corneometer measurements were well tolerated. Pigmentation status of lesions treated with were also performed early evening approximately 2 h after the PD P 506 A–PDT was considered ‘normal’ in the majority of third application of the day and there was then a final mea- cases (91% AK 03; 88% AK 04) and did not differ statistically surement on the morning of day 8. The mean hydration read- from those treated with placebo–PDT (P = 0Æ949; AK 04). ings before any treatment was applied (baseline) on day 1 However, in study AK 04 hypopigmentation after treatment were 34Æ54 ± 9Æ27 for DB gel, and 36Æ59 ± 10Æ23 for AC. On with cryosurgery was seen in 33% of lesions. Pigmentation the mornings of days 3, 5 and 8 the mean readings for DB status was found to be statistically significantly different gel were 39Æ98 ± 10Æ22, 44Æ16 ± 9Æ52 and 45Æ47 ± 7Æ63, between PD P 506 A–PDT and cryosurgery (P < 0Æ001). Fur- whereas the morning mean hydration readings for AC were thermore, 95% of patients were very satisfied or satisfied with 36Æ73 ± 8Æ1, 38Æ90 ± 11Æ45 and 36Æ29 ± 7Æ77, respectively. the overall cosmetic outcome of PD P 506 A–PDT as opposed By the evenings of days 1, 3 and 5 the mean results for DB to 82% after cryosurgery and 44% after placebo–PDT (AK gel were 48Æ28 ± 14Æ22, 49Æ85 ± 8Æ78, and 49Æ42 ± 8Æ32, 04). Patient’s as well as investigator’s assessment of the cos- respectively, whereas the corresponding mean hydration read- metic outcome of cleared lesions was significantly better for ings for AC were 43Æ80 ± 13Æ12, 41Æ14 ± 10Æ1 and 39Æ17 ± PD P 506 A–PDT than for cryosurgery (P < 0Æ001 for patient 8Æ39. These results demonstrate that although the corneometer

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 64 Bristol Cup Posters readings for both DB gel and AC taken immediately before the P-87 first application of the day were lower than their maximum Audit on prescribing and monitoring of methotrexate values reached by the end of the previous day of treatment, in G. Kaushal, E. Davies and S.A. Grabcyznska the case of the DB gel readings they showed a significant Amersham Hospital, Amersham, U.K. increase over time when compared with baseline, whereas in The BAD issued clinical guidelines on the prescribing of meth- the case of AC the readings returned to their pretreatment otrexate for patients with psoriasis (www.bad.org.uk/health baseline each morning. This difference in favour of DB gel care/guidelines/psormetho.asp [accessed 25 Feb 08]). The was statistically significant for all time points (Wilcoxon’s guidelines state features which should be addressed when ini- rank-sum test, P < 0Æ05) with the exception of the day 3 tiating and monitoring treatment with methotrexate. A review morning measurement (P = 0Æ09). This study demonstrates of clinical practice of local dermatologists was undertaken by that DB gel provides significantly better cumulative skin hydra- a questionnaire and case note audit. The aim was to identify tion than AC. how close the actual prescribing practice was to the recom- mended guidelines, and also to see how well this information was documented. Seventeen out of 25 dermatologists returned completed questionnaires. Fifty-three case notes were reviewed of patients prescribed methotrexate for treatment of P-86 psoriasis. Of the 17 dermatologists all gave positive answers to Unsuccessful treatment of extramammary Paget’s the questions on enquiring about past medical history, alcohol disease with topical imiquimod history, drug history and issues relating to family planning J. Gass, E. Rytina, J. Sterling and P.M. Todd and contraception. On review of the case notes, 38% had doc- Addenbrookes Hospital, Cambridge, U.K. umentation of a past medical history, 38% had recorded the Extramammary Paget’s disease of the anogenital area is an drug history and 34% had mentioned the alcohol intake and uncommon neoplasm, associated with a high incidence of only 11% had made reference to any issues relating to contra- underlying in situ or invasive malignancy. Treatment options ception or family planning. All dermatologists claimed to include Mohs’ micrographic surgery, wide surgical excision check pretreatment FBC, U&E and LFTs; 85% of case notes and radiotherapy, although the recurrence rate post-treatment had evidence of this. Seventy per cent of dermatologists is high. Recently, although there have been multiple reports claimed to check a pretreatment PIIINP level; 51% of the notes of topical treatment with imiquimod resulting in clinical and had evidence of this. Ninety-four per cent of dermatologists histological cure (Cohen PR, Schulze KE, Tschen JA et al. Treat- said they provided written information on methotrexate and ment of extramammary Paget disease with topical imiquimod 82% provided a monitoring booklet to the patient, yet only cream: case report and literature review. South Med J 2006; 99: 30% of the case notes had documentation of written informa- 396–402; Zampogna JC, Flowers FP, Roth WI, Hassenein AM. tion being given, and 28% of a monitoring booklet being pro- Treatment of primary limited cutaneous extramammary Paget’s vided. Seventy-six per cent of the dermatologists initially gave disease with topical imiquimod monotherapy: two case a test dose of methotrexate, this varied between 2Æ5 and 6Æ5 reports. J Am Acad Dermatol 2002; 47 (4 Suppl.): S229–351), mg; 74% documented this in the notes. Sixty-five per cent of reports of the failure of imiquimod therapy are lacking. We dermatologists claimed to prescribe folic acid with regimes describe the cases of two women presenting with vulval irrita- varying from 5 mg weekly to 10 mg three times weekly. This tion who had biopsy-proven extramammary Paget’s disease was only documented in 34% of the notes. Ninety-four per of the vulva treated within the last 12 months with topical cent of dermatologists performed 2–3-monthly blood tests imiquimod. Both underwent extensive investigations which once methotrexate had been initiated, 92% of case notes had revealed no underlying neoplasm, although one woman had documentation of these blood tests. Forty-seven per cent of previously treated carcinoma of the breast. Despite treatment 3 dermatologists admitted to checking PIIINP levels between 3 times weekly for more than 4 months, both had persistent and 6 months after initiating treatment, yet there was a mea- disease on examination and repeat biopsy, although clinically surement in 64% of the case notes. Even though the majority both had shown improved symptoms with lessening of irrita- of dermatologists practise in line with the recommended tion. Both women are currently being assessed for further guidelines, the documentation in the case notes does not fully treatment. These two cases illustrate that imiquimod treatment reflect this. We propose a tick-box proforma to be introduced may not be universally successful for extramammary Paget’s which would be incorporated into the case notes as proof of disease, and may thus delay definitive treatment. Patients trea- practice of all areas in the prescribing and monitoring of ted with imiquimod should be kept under close review to methotrexate. monitor both clinical and histological response to treatment. Furthermore, controlled trials are needed to assess the appro- priate use of imiquimod for this condition.

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 65

P-88 double-blind, randomized, placebo-controlled, parallel-group Nicorandil causes peristomal ulceration clinical trial designed to evaluate the efficacy and safety of a J. Donaldson, C. Flohr and J.S.C. English combination of acitretin (25 mg) and pioglitazone (15 mg) in Queens Medical Centre, Nottingham, U.K. patients with severe plaque-type psoriasis. The study was car- The potassium channel activator nicorandil, used in the treat- ried out in 41 patients with severe chronic plaque-type psoria- ment of angina, can induce oral and perianal ulceration. Such sis after due approval by the Institutional Ethics Committee. ulcers are granulomatous, sometimes painful and only resolve Patients of either sex in the age range of 18–65 years having a after discontinuation of the drug. We present two cases of peri- body surface area (BSA) involvement of > 20% were included. stomal ulceration induced by nicorandil to further support recent The patients were excluded if they were women of childbear- reports of these associations (Ogden S, Mukasa Y, Lyon CC, ing potential, had impaired liver or renal function tests, hyper- Coulson IH. Nicorandil-induced peristomal ulcers: is nicorandil lipidaemia, body mass index > 30 kg m–2, or were diabetic or also associated with the gastrointestinal fistula formation? Br J had coronary artery disease. After obtaining written informed Dermatol 2007; 156: 608–9; Claeys A, Weber-Muller F, Trechot consent, 22 patients were randomly assigned to the acitretin + P et al. Cutaneous, perivulvar and perianal ulcerations induced placebo group while 19 patients were assigned to the acitretin by nicorandil. Br J Dermatol 2006; 155: 494–6). Patient 1 was a + pioglitazone group. The study schedule was: screening visit, 73-year-old man who had a trephine transverse loop colostomy followed by single-blind, 2-week, placebo run-in period and for sigmoid adenocarcinoma. Two weeks after nicorandil (20 subsequently double-blind, 12-week study period. Post-ran- mg daily) was started for angina he developed a peristomal domization, patients were followed at 2, 4, 8 and 12 weeks. ulcer. Four treatments with cryotherapy over 3 months were At each follow-up visit the participants were evaluated for effi- unsuccessful. A biopsy found only granulation tissue. Following cacy, safety and compliance. Efficacy was evaluated by observ- a dermatological review, nicorandil was stopped, and the ulcer ing change in Psoriasis Area and Severity Index (PASI) score. resolved within 6 weeks. Patient 2 was a 73-year-old lady who The data analysis was intention-to-treat. Continuous variables was receiving nicorandil (60 mg daily) for angina, when she were analysed using the Mann–Whitney U-test while categori- underwent a Hartmann’s rectosigmoidectomy with colostomy cal variables were analysed using the v2 or Fisher’s exact test. for bowel perforation complicating diverticulitis. She remained A P-value < 0Æ05 was considered as significant. The two treat- on nicorandil 60 mg daily for 2 years postoperatively, before ment groups were comparable in baseline variables. After 12 developing a peristomal ulcer. On dermatological referral nico- weeks of therapy, the reduction in mean PASI score was signif- randil was stopped. Three months later, the peristomal ulcer icantly greater in the acitretin + pioglitazone group [mean ± had completely healed. The aetiology of nicorandil-associated SD 6Æ0 ± 5Æ6 (median, range 4Æ8, 1Æ1–24Æ3)] in comparison to ulceration is yet undetermined. Postulated mechanisms include the acitretin + placebo group [10 ± 8Æ5 (8Æ0, 0Æ9–39Æ1); P = a ‘vascular steal’ phenomenon or direct action of nicorandil or 0Æ045]. At the end of 12 weeks, the percentage reduction in one of its metabolites. Nicorandil ulceration is postulated to be PASI score was 64Æ2% (95% CI 49Æ2–79Æ3) in the acitretin + more frequent following dose increases and where higher doses pioglitazone group compared with 51Æ7% (95% CI 38Æ7–64Æ7) (40–60 mg daily) are used, although one of our patients devel- in the acitretin + placebo group. The majority of adverse oped ulceration at 20 mg daily. Nicorandil-induced ulceration events were not serious except for one possibly unrelated epi- may have a high morbidity and failure to recognize this associa- sode of acute myocardial infarction in a 49-year-old woman. tion could lead to inappropriate management. Pioglitazone may provide a convenient, efficacious, and rela- tively safe drug for combining with acitretin as an alternative to other currently available more toxic antipsoriatic agents. The study was an investigator-initiated study with no fund- ing from any pharmaceutical company. P-89 Efficacy and safety of acitretin plus pioglitazone vs. acitretin plus placebo in patients with severe plaque-type psoriasis: a randomized, double-blind, parallel-group clinical trial P-90 R. Mittal, P. Pandhi, I. Kaur, S. Dogra and The use of topical dimethyl sulphoxide (DMSO) S. Malhotra in Jessner’s lymphocytic infiltrate PGIMER, Chandigarh, India K. Panting and R.M. Azurdia Disordered differentiation and hyperproliferation of keratino- Broadgreen Hospital, Liverpool, U.K. cytes with underlying inflammation are hallmarks of psoriasis. We report the successful treatment with topical dimethyl Agonists of peroxisome proliferator-activated receptor (PPAR)- sulphoxide (DMSO) of three cases of Jessner’s lymphocytic have been demonstrated to normalize the histological fea- c infiltrate that have all been resistant to multiple therapies in the tures of psoriasis. Thus, a combination of PPAR- agonists with c past. Jessner’s lymphocytic infiltrate is a chronic benign lym- retinoids may enhance the efficacy of retinoids (acitretin) in phocytic infiltrate of exposed skin particularly the head, neck, the therapy of psoriatic patients. The present study was a V-chest and upper back. It presents with persistent red plaques

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 66 Bristol Cup Posters or tumid nodules and is often resistant to treatment but can 53–5) but there are few data on the efficacy of narrow-band resolve spontaneously after months or years. There is very little ultraviolet B (TL01 UVB) although this form of phototherapy evidence basis for successful treatments, with topical steroids has been increasingly used to treat this condition. An audit of tending to be first-line therapy. Other listed treatments with patients with prurigo treated with phototherapy in our derma- varying degrees of evidence include topical calcineurin inhibi- tology unit between January 2000 and January 2007 was car- tors, oral antimalarials, etretinate, thalidomide, PUVA, aurano- ried out. The aim was to assess the response of patients fin, radiotherapy and dapsone. The index case heard about the treated with TL01 UVB and PUVA. Treatment regimes con- use of DMSO via an internet patient forum and ordered the sisted of a starting dose of 70% of the minimal erythema dose treatment from the USA privately. The two other cases were (MED) for TL01 UVB patients and 70% of the minimal photo- informed of this success and took it upon themselves to order toxic dose (MPD) for patients treated with PUVA. Incremental the same product with similar beneficial results. All three cases increases of 20% were then given at each visit adjusted have had a dramatic response to DMSO with the effects being according to the erythema response. The outcome was sustained with little in the way of side-effects other than some assessed under the supervision of the consultant dermatologist mild skin irritation and a garlic-like odour. DMSO is a small responsible for the phototherapy service as clear, minimal molecule which is a by-product of the wood industry and a residual activity (MRA), good improvement or poor outcome known irritant, with antioxidant properties as a potent scaven- (moderate, mild or no benefit). Data were collected from gen- ger of free radicals. It is used as a vehicle for the topical appli- eral and phototherapy case notes as well as a phototherapy cation of some pharmaceuticals as it is thought to increase the database. A total of 44 patients (15 males and 29 females) transmembrane passage of certain drugs; historically it has also received a course of TL01 UVB and 18 patients (8 males and been used in photodynamic therapy as a penetration enhancer 10 females) were treated with PUVA. The median age of and as a cryoprotectant. The U.S. Food and Drug Administra- patients receiving TL01 UVB was 55 years [interquartile range tion (FDA) has approved DMSO usage only for the palliative (IQR) 40–65] and the median age of patients receiving PUVA treatment of interstitial cystitis, as a 50% solution, and as a pre- was 58 years (IQR 52–72). The distribution of subacute or servative of organs for transplant. It is also commonly used in chronic prurigo between the groups was similar. The median the veterinary field for equine musculoskeletal injuries as a number of exposures in the PUVA-treated group was 23 (IQR potent anti-inflammatory agent. To our knowledge there have 20–29) with a median cumulative dose of 111Æ74 J cm–2 been no reported cases of Jessner’s lymphocytic infiltrate trea- (IQR 73Æ9–154Æ58). The median number of exposures in ted with topical DMSO, although there have been a huge vari- patients receiving TL01 UVB was 30 (IQR 27–39) with a ety of uses over the years with many reports, including the median cumulative dose of 43309Æ5 mJ cm–2 (IQR 24033– successful treatment of scleromyxoedema, cutaneous amyloid, 55047). Sixteen of the 18 patients (89%) treated with PUVA psoriasis, herpes simplex virus infection, chemotherapy-related achieved a good outcome (clear, MRA or good benefit) com- skin necrosis and even prevention of surgical flap necrosis. One pared with 30 (68%) of the 44 patients in the TL01 UVB is hesitant to recommend the use of an unlicensed product group. More patients in the PUVA group experienced side- until perhaps further studies into the mechanism of action have effects. This audit suggests a greater effectiveness of PUVA been done, to evaluate its role as well as the short- and long- compared with TL01 UVB as a treatment for prurigo. This term safety of using DMSO in localized skin disease – is it may relate to the ability of longer wavelength UVA to pene- worth re-visiting this old compound? trate more deeply into the acanthotic and hyperkeratotic epi- dermis characterizing prurigo lesions. A randomized controlled trial is necessary to confirm these findings.

P-91 Audit of phototherapy in the treatment of subacute and chronic prurigo P-92 D. Turner, B. Rathmell and V. Goulden Marked long-term clinical response to treatment Dermatology Department, The General Infirmary at Leeds, Leeds, U.K with ustekinumab, a new monoclonal antibody for Subacute and chronic prurigo is characterized by persistent the treatment of psoriasis pruritic papules and nodules frequently located on the exten- A. Kimball, C. Leonardi,* K. Papp, N. Yeildingà and sor surfaces of the limbs. The condition is often resistant to K. Gordon§ topical therapy. Systemic treatment, in particular immunosup- Harvard Medical School, Boston, MA, U.S.A.; *St Louis University Medical pression, is generally more successful but associated with School, St Louis, MO, U.S.A.; Probity Medical Research, London, Ontario, potentially serious adverse effects. The role of photochemo- Canada; àCentocor Research and Development, Inc., Malvern, PA, U.S.A. therapy (psoralen plus ultraviolet A; PUVA) in the treatment and §Northwestern University Feinberg School of Medicine, Skokie, IL, of prurigo is well established (Karvonen J, Hannuksela M. U.S.A. Long term results of topical trioxsalen PUVA in lichen planus Ustekinumab (UST; CNTO-1275) demonstrated significant and nodular prurigo. Acta Derm Venereol (Stockh) 1985; 120: efficacy in patients with moderate-to-severe plaque psoriasis in

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 67 a short-term, phase II study. A high proportion of responders P-93 had a marked response as measured by Psoriasis Area and Regional audit of morphoea management Severity Index (PASI) 90 or complete clearing of their psoria- A.S. Alkali, A. Khalid and J.M. Sowden* sis as measured by PASI 100 or a Physician’s Global Assess- Department of Dermatology, Broadgreen Hospital, Liverpool, U.K. and ment (PGA) of cleared. We report on the efficacy of UST in *Department of Dermatology, Wrexham Mealor Hospital, Wrexham, U.K. maintaining marked responses with long-term maintenance Morphoea is a connective tissue disease that affects the skin therapy. PHOENIX 1 was a phase III, double-blind, placebo- and the subcutaneous tissues. Occasionally underlying struc- controlled study of 766 patients randomized to UST (45 or tures such as muscle, fascia and bone may be involved. Mor- 90 mg s.c. doses at weeks 0 and 4 followed by 45 or 90 mg phoea may cause serious physical disability due to secondary every 12 weeks) or placebo. Patients in the placebo group muscle atrophy and interference with bony growth and also crossed over in equal proportions to 45 or 90 mg doses at serious cosmetic defects due to skin atrophy and pigmentary weeks 12 and 16, then every 12 weeks. The efficacy of long- changes. Although many treatments are used none is generally term maintenance therapy was evaluated using a randomized accepted as being reliably effective. A regional audit was per- withdrawal design, in which long-term PASI 75 responders formed assessing the management of morphoea in comparison were randomized at week 40 to continue maintenance dosing with published evidence. A questionnaire survey of 35 regio- with UST or to withdraw from therapy and receive placebo. nal dermatologists and a case note review of 70 patients with Patients randomized to placebo were retreated after loss of ‡ morphoea across four hospitals were conducted. Completed 50% improvement in PASI. During the randomized with- questionnaires were received from 28 (80%) of the 35 derma- drawal period, significantly higher proportions of patients tologists (15 consultants, 2 associate specialists, 9 specialist continuing UST maintained PASI 75 compared with patients registrars, 1 staff grade and 1 trust grade). From the question- withdrawn from therapy (P < 0Æ001). At week 52, 87% and naire survey, investigations considered appropriate are skin 91% of patients who continued 45 and 90 mg, respectively, biopsy 23 of 28 (82%), FBC 22 of 28 (79%), ESR 20 of 28 were PASI 75 responders compared with 64% and 62% of (71%), protein electrophoresis 11 of 28 (39%), ANA/ENA 27 patients withdrawn from therapy (P £ 0Æ001, each compari- of 28 (96%), X-ray 1 of 28 (4%) and Borrelia serology 9 of 28 son). At week 40, 70% and 73% of patients (combined 45 (32%). Treatments considered to be appropriate for first-line and 90 mg maintenance and withdrawal groups, respectively) use were topical steroids 28 of 28 (100%), intralesional ste- who entered the randomized withdrawal phase were PASI 90 roids 5 of 28 (18%), topical calcipotriol 16 of 28 (57%), responders; PASI 100 was achieved by 38% and 42% of betamethasone 0Æ05% ⁄calcipotriol ointment 10 of 28 patients and PGA of cleared was achieved by 44% and 48% of (36%), topical imiquimod 4 of 28 (14%), and topical tacroli- patients in the maintenance and withdrawal groups, respec- mus 10 of 28 (36%). Treatments considered appropriate for tively. At weeks 52 and 76, respectively, 65% and 57% of second-line use were systemic PUVA 22 of 28 (79%), topical patients receiving maintenance therapy achieved PASI 90 com- PUVA 23 of 28 (82%), UVA1 7 of 28 (25%) and physiother- pared with 38% and 6% of patients withdrawn from therapy; apy 13 of 28 (46%). Treatments considered for third-line use corresponding PASI 100 responses were 40% and 34% of were methotrexate 27 of 28 (96%), methotrexate plus pulsed patients receiving maintenance dosing and 14% and 3% of prednisolone 15 of 28 (54%), oral calcipotriol 5 of 28 (18), patients withdrawn from therapy. PGA of cleared was achieved ciclosporin 20 of 28 (71%) and surgery 2 of 28 (7%). From by 44% and 40% of patients receiving maintenance dosing the case note audit the 70 patients consisted of 58 females compared with 16% and 4% of patients withdrawn from ther- (83%) and 12 males (13%). There was good documentation apy. Continuous maintenance therapy with UST was generally of history and examination findings; however inadequacies well tolerated. During the randomized withdrawal period, were noted with documentation of Raynaud’s phenomenon, 67% of patients continuing UST experienced ‡ 1 adverse dysphagia and history of borreliosis or foreign travel. Investi- events (AE) compared with 76% of patients withdrawn from gations requested were as follows: skin biopsy (60 patients), therapy; 1Æ9% of patients in each group experienced an AE FBC (33 patients), ESR (26 patients), protein electrophoresis leading to therapy discontinuation. The proportion of patients (11 patients), ANA/ENA (36 patients), X-ray (8 patients) and with a serious AE was 0Æ6% and 4Æ4% for the maintenance Borrelia serology (9 patients, of which one was positive). and withdrawal groups, respectively. With long-term Response rates to first-line treatment were topical steroids 26 continuous therapy, marked responses to UST remained gener- of 53 (49%), intralesional steroids 1 of 1 (100%), betametha- ally stable over time. Long-term therapy was generally well sone 0Æ05% ⁄calcipotriol ointment 2 of 4 (50%), calcipotriol 1 tolerated. of 7 (14%), imiquimod 1 of 1 (100%) and topical tacrolimus This study was supported by Centocor Research and Devel- 3 of 8 (38%). The response rates to second- and third-line opment, Inc. treatments were systemic PUVA 1 of 5 (20%), topical PUVA 1 of 1 (100%), methotrexate 5 of 6 (83%), methotrexate plus pulsed prednisolone 2 of 2 (100%), ciclosporin 2 of 4 (50%), physiotherapy 2 of 2 (100%) and surgery 1 of 1 (100%). Patient information leaflets were documented as being pro- vided to 13 of 70 patients. We conclude that treatment

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 68 Bristol Cup Posters policies in all Trusts closely match the published evidence. We response and experienced more side-effects. However, our recommended that the diagnosis of morphoea is clinical; how- data were similar to a review on FAE performed in a regional ever skin biopsy, FBC, ESR, ANA/ENA, and other investiga- centre in the U.K. (Harries MJ, Chalmers RJ, Griffiths CE. FAE tions including electrophoresis, Borrelia serology and imaging for severe psoriasis: a retrospective review. Br J Dermatol 2005; studies should be requested when appropriate. We plan to 153: 549–51). We conclude that FAE can be safe and effective re-audit our services in 2 years time to complete the audit cycle. agents in a selected cohort of severe recalcitrant psoriasis. Adverse effects were common, leading to cessation of its use in about a third of patients.

P-94 Fumaric acid esters: an audit on their efficacy in the U.K. P-95 V.H. Smith and P.D. Yesudian* A population study of common truncating polymor- Royal Liverpool & Broadgreen University Hospitals NHS Trust, Liverpool, phisms of profilaggrin and dry skin phenotype U.K. and *Countess of Chester Hospital, Chester, U.K. A. Sergeant, L.E. Campbell,* M. Porter, P.R. Hull,* Fumaric acid esters (FAE) have been used extensively in F.J.D. Smith,* W.H.I. McLean and C.S. Munro Northern Europe, particularly Germany, where they are rec- Alan Lyell Centre for Dermatology, Glasgow, U.K. and *Human Genetics ommended for severe plaque psoriasis refractory to conven- Unit, University of Dundee, Dundee, U.K. tional therapy. Their use in the U.K. is however unlicensed. The findings that ichthyosis vulgaris is caused by common We undertook this audit with BAD clinical guidelines (www. truncating polymorphisms of profilaggrin, precursor of the bad.org.uk/healthcare/guidelines/psorfumaric.asp [accessed cornified envelope protein filaggrin, and that heterozygosity 25 Feb 08]) as our standard. Eight hospital trusts in the for these polymorphisms is an important predisposing factor region were included in our audit. In three hospitals, FAE for eczema and other atopic diseases, represent a major were nonformulary drugs and unavailable. A retrospective advance in understanding these diseases. However, the poly- review of notes was performed in the other five hospitals. morphisms are common, being found in about 10% of the Patients were highlighted via pharmacy records. A total of 37 Scottish population. It has not been shown that they result in patients on FAE were identified (male : female 17 : 20) (age a clinical phenotype in all cases. To investigate the symptom- range 18–74 years). The diseases treated were chronic plaque atic and phenotypic effects of these variants in a population psoriasis (33), pustular psoriasis (2) and necrobiosis lipoidica not selected for symptoms of dry or inflamed skin or atopy, (2). Mean disease duration prior to FAE was 17 years (range we studied patients presenting to dermatology for diagnosis of 3–41 years). Previous treatments included phototherapy (29), skin lesions. Two groups were studied: subjects aged 18–40 inpatient therapy (18) and other systemic agents (35) (95%). years (n = 192, 82 male); and subjects aged 60–75 years (n = Thirty-two patients (91%) had tried two or more systemic 99, 55 male). Each was asked to complete a simple question- therapies. FAE were commenced in accordance with the incre- naire on symptoms of dry skin and history of skin or atopic mental dosage regime in all 37. Ten patients had FAE intro- disease, and scored for signs of ichthyosis at specific sites. duced overlapping another systemic agent, in four of which DNA samples were analysed for four profilaggrin polymor- this was a short-term manoeuvre. In the six others, the over- phisms known to occur in this population. In samples typed, lap was designed to facilitate a dose reduction of the other heterozygous status was confirmed for R501X in 15 of 285 therapy (successful in three). The maximum dose of 720 mg (5%), 2282del4 in 13 of 286 (4%), R2477X in 4 of 291 daily was reached in 18 patients. The rating of effectiveness (1%) and S3247X in 2 of 211 (0.9%), consistent with previ- was: ‘clear’ 4 (11%), ‘virtually clear’ 6 (16%), ‘some ous studies in this population. The majority of proximal poly- improvement’ 15, ‘no help’ 12. The mean response time was morphisms are predicted greatly to reduce filaggrin 12 weeks. Side-effects included abdominal pain (14), flushing expression. Three compound heterozygotes were identified: (13), nausea and vomiting (4), and none (12) (33%). Blood R501X/R2477X (2) and R2477X/S3247X (1). Carriers of one abnormalities were observed in 11 patients (mainly abnormal or more polymorphisms were more likely to report moder- LFTs, lymphopenia), all of which were transient. At the time ately or very dry skin [odds ratio (OR) 3Æ4; 95% CI 1Æ5–7Æ2], of completion of the audit, 14 were still on FAE and 23 had and use of body moisturizers twice a week or more (OR 2Æ1; discontinued therapy, reasons being side-effects (10), lack of 95% CI 1Æ0–4Æ6), but this was not significant in women, or efficacy (11) or both (2). The response rate to FAE was 27%, older subjects, alone. Carriage increased the likelihood of a with remission achieved within 12 weeks. More than 60% of history of childhood eczema (OR 3Æ1; 95% CI 1Æ3–7Æ7) but patients developed adverse effects. Compared with randomized not of other atopic diseases. It was not significantly associated controlled trials on FAE (Altmeyer PJ, Matthes U, Pawlak F et with self-reported ‘sensitive skin’, psoriasis or acne. In both al. Antipsoriatic effects of fumaric acid derivatives. Results of a age groups, heterozygotes were more likely to have visible multicenter double-blind study in 100 patients. J Am Acad Der- fine scaling (OR 3Æ2; 95% CI 1Æ4–7Æ4) or rough skin texture matol 1994; 30: 977–81), our cohort demonstrated a poorer (OR 2Æ5; 95% CI 1Æ1–5Æ5). Palmar hyperlinearity (OR 3Æ2;

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69 Bristol Cup Posters 69

95% CI 1Æ3–8Æ0) and roughness were also increased overall, use. The price of cinnamon has gone up by 30% over the last but a trend to increased keratosis pilaris was not significant. 18 months because not enough is being produced to meet While we have confirmed that heterozygosity for truncating demand. It looks likely that dermatologists are going to con- mutations increases the chance of ‘dry’ skin, the phenotype is tinue to see cinnamon-related contact dermatitis through con- not universal. Most older, but only half of younger, carriers of tinued use of this historic spice. truncating polymorphisms had any symptoms or signs. Con- versely, many subjects with wild-type profilaggrin at all four P-97 sites tested had symptoms or signs of dry skin. Additional fac- Frederick Parkes Weber 1863–1962: a gentleman tors which may contribute to this phenotype in the general and a scholar population and pedigrees of ichthyosis vulgaris may include S.D. Orpin and A.H.M. Heagerty unidentified polymorphisms, and variable expression of wild- Heart of England NHS Foundation Trust, Birmingham, West Midlands, U.K. type filaggrin or other cornified envelope proteins. The name Weber is well known to medical students and qual- ified doctors alike, particularly those from the dermatology fraternity, as part of numerous eponymous syndromes. The P-96 man behind the names of a collection of uncommon congeni- Cinnamon: a spice with history tal and inherited disorders is little known today but well in to D.J. Eedy his nineties he corresponded to medical journals and contrib- Craigavon Area Hospital Group Trust, Portadown, U.K. uted original articles to them and was considered the leading Cinnamates are an important allergen in contact dermatitis authority on rare medical disorders of his day. His many aca- occurring in a wide range of products from perfumes, cosmet- demic exploits were not limited to medical subjects alone. It ics, personal hygiene products to drinks such as cola, mouth- is perhaps not surprising that the eldest son of Sir Hermann washes and toothpastes. Cinnamon has been known from Weber, a renowned physician in the latter part of the 19th antiquity and was imported to Egypt from China as early as century who had tended to no less than five Prime Ministers 2000 BC. It was used in Old Testament times. It was com- as well as Queen Victoria, should himself become an equally monly used on funeral pyres in Rome and the Emperor Nero prominent medical figure. He studied at Trinity College, is said to have burned a year’s supply of cinnamon at the fun- Cambridge before completing his training at St Bartholomew’s eral for his wife Poppaea Sabina in AD 65. In the early days, Hospital. In 1894 he was appointed physician to the German it was collected in the wild in Ceylon and Indonesia. Portu- Hospital, Dalston, a post that had also been his father’s and guese traders discovered Ceylon at the end of the 15th cen- which he was to hold for 50 years. Although primarily a gen- tury, and restructured the traditional production of cinnamon. eral physician Parkes Weber devoted a good deal of his medi- The Portuguese established a fort on the island in 1518, and cal energies to dermatology, about a quarter of his enormous protected their own monopoly for over a hundred years. From number of citations pertaining to this subject. He was the first the 16th to the 18th centuries cinnamon was the ‘Holy Grail Mitchell lecturer to the Royal College of Physicians in 1921 of foreign invaders’. After the coastal provinces were con- before being awarded the Moxton gold medal for distin- quered by the Dutch East India Company, small commercial guished observation and clinical research in 1930. He also groves were planted in the late 18th century. It became one found time to study pathology becoming a member of the of the main exports of the Dutch East India Company, and the Pathological Society of London in 1894 and contributing to its subject of protracted and expensive wars between Portugal Journal as late as 1961. His interests beyond the medical and Holland. While Sri Lanka’s most famous export is tea, his- sphere are too many to list in detail but it seems whatever torically the most important has been cinnamon. Today Sri subject he turned his scholarly mind to he pursued with great Lanka is one of the world’s leading exporters of cinnamon, energy and enthusiasm. An avid collector of antiquities of all producing about four-fifths of international output. In fact, so sorts he was interested in coins, particularly those from strong is the link between cinnamon and Sri Lanka that the ancient Greece. He was made an honorary fellow of the Royal botanical name of the spice – Cinnamomum zeylanicum – is Numismatic Society and he even endowed a prize, the Parkes derived from the island’s former name, Ceylon. Cinnamon Weber medal, which is still awarded today. He even wrote on trees have a productive life span of about 40–50 years after philosophical subjects and in 1910 published a book entitled which they have to be replanted. The plant grows as a bush Aspects of Death and Correlated Aspects of Life in Art, Epigram and Poetry, and every morning cinnamon peelers go to the fields to collect the fourth edition of which appeared in 1922 and was dedi- branches to be stripped for the thin inner bark strips that curl cated to Sir William Osler no less. Frederick Parkes Weber was into rolls (quills) on drying. Sri Lanka’s cinnamon groves lie a physician with a broad range of clinical knowledge and mostly in its western and south-western regions, north and experience and an encyclopaedic memory. He was also a poly- south of the capital, Colombo. The tropical sunshine and math with extensive interests in many other learned pursuits. abundant rain in these areas provide an ideal habitat, but even A gentle and courteous man he was always willing to help here the quality of the spice varies with soil conditions. The others and at the end of his long life he had more than a growing and collection of cinnamon is labour intensive and thousand citations on diverse matters medical and otherwise. its world consumption is rising rapidly for increasing culinary Truly, he was a gentleman and a scholar.

Ó 2008 The Authors Journal Compilation Ó2008 British Association of Dermatologists • British Journal of Dermatology (2008) 159 (Suppl. 1): 24–69