Acta Derm Venereol (Stockh) 1998; 78: 220–239

LETTERS TO THE EDITOR

Juvenile Generalized Pustular Psoriasis

Sir, The mixed type has both Zumbusch and annular patterns. Generalized pustular psoriasis refers to a group of disorders The case presented fits best into the mixed type of generalized characterized by a widespread eruption of sterile pustules. In pustular psoriasis. Various triggering factors have been identi- 1910 von Zumbusch described a patient who had had psoriasis fied, including infection, vaccination and steroids (5). Juvenile vulgaris from childhood and who later developed generalized generalized pustular psoriasis can occur at any age, but the pustulation (1). He observed the patient through nine episodes onset of the disease is often during the first year of life (2, 3). of generalized pustulation, spanning 10 years. Generalized Unlike other forms of psoriasis, males are more affected than pustular psoriasis is extremely rare in childhood; only 100 females (sex ratio 352) (2, 6). cases are reported in the literature (2, 3). In their series of 104 The differential diagnosis of childhood generalized pustular cases of generalized pustular psoriasis, Baker & Ryan observed psoriasis includes staphylococcal scalded skin syndrome, pityri- only 5 children (4). asis rubra pilaris, toxic epidermal necrolysis, erythrodermic psoriasis, Reiter’s disease, generalized candidiasis and general- CASE REPORT ized atopic dermatitis or seborrhoeic dermatitis (7). The treatment of generalized pustular psoriasis in childhood An 11-year-old boy presented with an 8-year history of a recurrent is difficult. Regarding the benign course of the disease in most ff generalized pustular eruption. He su ered from fever, malaise and of the cases, initial treatment should be topical, e.g. topical pain. On examination there were pustules on an erythematous basis, steroids are useful. spreading all over the body. The pustules in some areas coalesced to form lakes (Fig. 1). Additionally, we found scattered erythematous In cases associated with severe systemic symptoms, oral plaques arranged in an annular pattern on the upper limbs and the etretinate is the treatment of choice (8, 9). Systemic steroids trunk. The plaques were covered with superficial pustules. Laboratory were not helpful (7). Methotrexate should only be used in evaluation revealed an increased leukocyte count of 28×109/l. children in very exceptional cases like universal pustular Treatment with acitretine (NeotigasonA), 30 mg/day, was given, psoriasis (6). Hydroxyurea, PUVA and cyclosporin A are and an improvement occurred in the next 5 weeks. A massive usually not recommended in children. desquamation of the whole skin preceded the healing. REFERENCES DISCUSSION 1. Zumbusch L. Psoriasis und pustulo¨ses Exanthem. Arch Dermatol Psoriasis vulgaris occurs in more than 59% of patients with Syph 1910; 99: 335–346. generalized pustular psoriasis, and 25% have a positive family 2. Beylot C, Bioulac P, Grupper C, Desmons F, Larregue M, history (2). In a report of 27 children, Beylot et al. divided Maleville J. Generalized pustular psoriasis in infants and children. juvenile generalized psoriasis into 3 groups: Zumbusch pattern, Report of 27 cases. In: Farber EM, Cox AJ, Jacobs PH, Nall L, eds. Psoriasis: Proceedings of the 2nd International Symposium. annular pattern and mixed type (2). The Zumbusch pattern New York: Yorke Medical Books; 1977. p. 171–179. is characterized by waves of widespread eruption of sterile 3. Beylot C, Puissant A, Bioulac P, Saurat JH, Pringuet R, Doutre pustules, associated with constitutional symptoms such as high MS. Particular clinical features of psoriasis in infants and children. fever, malaise, anorexia and pain. Most patients with this Acta Derm Venereol (Stockh) 1979; Suppl 87: 95–97. pattern tend to develop psoriasis vulgaris. The annular pattern 4. Baker H, Ryan TJ. Generalized pustular psoriasis: a clinical and is a more frequent, subacute eruption and is characterized by epidemiological study of 104 cases. Br J Dermatol 1968; 80: erythema and pustules in a circinate pattern. This form can 771–793. follow or precede the generalized form of pustular psoriasis. 5. Barta U, Wollina U. Psoriasis pustulosa. In: Wollina U, Hein G, Knopf B, eds. Psoriasis und Gelenkerkrankungen. Jena, Stuttgart: Gustav Fischer Verlag; 1996. p. 43–47. 6. Nyfors A. Psoriasis in children: characteristics, prognosis and therapy. A review. Acta Derm Venereol (Stockh) 1981; Suppl 95: 47–53. 7. Zelickson BD, Muller SA. Generalized pustular psoriasis in child- hood. Report of thirteen cases. J Am Acad Dermatol 1991; 24: 186–194. 8. Rosinska D, Wolska H, Jablonska S, Konca I. Etretinate in severe psoriasis in children. Pediatr Dermatol 1988; 5: 266–272. 9. Shelnitz LS, Esterly NB, Honig PJ. Etretinate therapy for general- ized pustular psoriasis in children. Arch Dermatol 1987; 123: 230–233.

Accepted October 20, 1997. Theodor Karamfilov, M.D. and Uwe Wollina, M.D.* Department of Dermatology, Friedrich Schiller University of Jena, Erfurterstr. 35, D-07740 Jena, Germany.

Fig. 1. Confluent pustulosis. * Address for offprints.

Acta Derm Venereol (Stockh) 78 © 1998 Scandinavian University Press. ISSN 0001-5555 Letters to the Editor 221

Eosinophilic Pustular Folliculitis Induced after Prolonged Treatment with Systemic Corticosteroids in a Patient with Pustulosis Palmoplantaris

Sir, Eosinophilic pustular folliculitis (EPF), originally described by Ofuji et al. in 1970 (1), is characterized by recurrent crops of pruritic follicular papules and pustules that occur mainly on the face, trunk, and extremities. We report here a patient with pustulosis palmoplantaris (PPP) who eventually developed EPF after prolonged treatment with systemic corticosteroids.

CASE REPORT

A 72-year-old Japanese woman presented with a recurrent pruritic, pustular eruption on the palms and soles: the eruption began in November 1994 and she was referred to our University Hospital in July 1995. Examination revealed scaly erythematous patches with pustules on the palms and soles. A diagnosis of PPP was made and Fig. 1. Clinical course in relation to sequential eosinophil counts and laboratory investigations showed a peripheral white blood cell count treatment modalities. Eosinophil counts increased coincidentally with of 6,700/ml. Therapeutic trials with oral antibiotics, antihistamines, exacerbation of the lesions after treatment with systemic corticoster- and topical corticosteroids were without benefit; fresh pustules con- oids, and rapidly returned to normal levels after starting indomethacin. tinued to appear on the palms and soles. Oral prednisone (20 mg day−1) was initiated and resulted in resolution of the lesions. However, when the dose of prednisone was tapered to 15 mg day−1, cultures, and the rapid response to indomethacin point to the the palmoplantar lesions recurred; the maintenance dose of diagnosis of EPF. 15 mg day−1 brought some resolution of the lesions, but her eruption Palmoplantar pustular or vesicular lesions have often been still had a tendency to reappear. The eruption kept recurring for 9 regarded as an early or abortive form of EPF that has months despite treatment with systemic corticosteroids. In April 1996, eventually evolved into typical EPF (2). Thus, EPF started as 6 months after starting oral prednisone, we recognized the pruritic PPP-like lesions in 8% of Japanese cases and the mean time erythematous lesions on her face. Pruritic erythematous papules interval between the onset of PPP-like lesions and the develop- developed on her trunk and extremities in May. At this time, the absolute eosinophil count had increased (1895/ml ) compared with that ment of the typical EPF lesions in the extrapalmoplantar before corticosteroid therapy (368/ml ). All these data suggested the regions was 26 months (2). We found that four cases, including diagnosis of EPF. HIV antibody was negative. Oral prednisone was our own one, had been treated with systemic corticosteroids discontinued. In June, many pruritic warts were noted on the trunk without success: most of these cases treated with systemic and extremities. corticosteroids developed EPF within 6 months after starting Histopathological findings of a biopsy specimen from the pustule systemic corticosteroids, as in our case; the mean age of the 4 on the left palm showed the presence of a pustule containing neutro- cases was 40 years, whereas that of the remaining 12 cases phils and eosinophils, spongiosis of the epidermis, and a dense that had not been treated with systemic corticosteroids was 51 eosinophilic and neutrophilic infiltrate located mostly around the years. These results suggest that systemic corticosteroids may pustule; numerous eosinophils with degranulation were also noted in act as a trigger for the development of EPF in predisposed the dermis. A diagnosis of EPF was made; and indomethacin (75 mg day−1) was begun in September 1996. In 2 weeks, the pustular individuals, particularly younger ones. lesions had disappeared, all except for warts. Eosinophil count (371/ml) Although the pathogenesis of EPF is not known, there can dramatically decreased after indomethacin therapy was started be a relationship between immunologic alterations including (Fig. 1). The patient has been seen regularly every 2 weeks since then, HIV infection and the development of EPF (3, 4). One possible and the dose of indomethacin has been tapered to 25–50 mg day−1. explanation could be a shift in the cytokine responses toward She remained disease-free with the maintenance dose of a Th2 state in such patients (5). Other factors that promote a 25–50 mg day−1 in an intermittent fashion. Th2 response are aging, stress and drugs such as corticosteroids (6). This can explain why in previous Japanese cases aged DISCUSSION patients with PPP tended to develop EPF over a prolonged period of time without corticosteroids. Thus, PPP and EPF We have reported a patient with palmoplantar lesions who should not be regarded as a static disease but as an extremely initially presented with clinical features typical of PPP and dynamic condition in which modulation of the cytokine eventually developed EPF. In this patient, oral corticosteroids responses induced by treatment modalities alters the outcome for 6 months apparently aggravated the lesion, which was of the disease. accompanied by absolute peripheral hypereosinophilia. It could be argued that the pruritic pustules we observed in this patient are somewhat different in clinical presentation from REFERENCES those in typical EPF originally described by Ofuji et al. (1): 1. Ofuji S, Ogino A, Horio T, Ohseko T, Uehara M. Eosinophilic annular erythematous plaques with vesiculopustular margins pustular folliculitis. Acta Derm Venereol (Stockh) 1970; 50: were not observed in this patient. However, the histologic 195–203. pattern, absolute peripheral eosinophilia, negative bacterial 2. Aoyama H, Tagami H. Eosinophilic pustular folliculitis starting

Acta Derm Venereol (Stockh) 78 222 Letters to the Editor

initially only with palmoplantar pustular lesions. Dermatology 6. Ramirez F, Fowell DJ, Puklavec M, Simmonds S, Mason D. 1992; 185: 276–280. Glucocorticids promote a Th2 cytokine response by CD4 T cells in 3. Patrizi A, Di Lernia V, Neri I, Gherlinzoni F. Eosinophilic pustular vitro. J Immunol 1996; 156: 2406–2412. folliculitis (Ofuji’s disease) and non-Hodgkin lymphoma. Acta Derm Venereol (Stockh) 1992; 72: 146–147. 4. Boone M, Dangoisse C, Andr J, Sass U, Sonog M, Ledoux M. Eosinophilic pustular folliculitis in three atopic children with hyper- Accepted November 17, 1997. sensitivity to dermatophagoides pteronyssinus. Dermatology 1995; 190: 164–168. Yoshiko Mizukawa and Tetsuo Shiohara 5. Clerici M, Shearer GM. Th1Th2 switch is a critical step in the Department of Dermatology, Kyorin University, School of Medicine, etiology of HIV infection. Immunol Today 1993; 14: 107–115. 6-20–2, Shinkawa, Mitaka, Tokyo, 181, Japan.

Acute Generalized Exanthematous Pustulosis Associated with Paracetamol

Sir, Acute generalized exanthematous pustulosis (AGEP) was named by Beylot et al. in 1980 (1) and its diagnostic criteria were established by Roujeau et al. (2). Drugs have been the causative agents of AGEP in most cases reported in the literature, particularly antibiotics. Cutaneous reactions with paracetamol are rare. The most common cutaneous side-effects are acute hypersensitivity or fixed drug eruptions, and occasionally eczema or vasculitis. Three cases of AGEP associated with paracetamol have recently been described (2–4). We here report 2 new cases of AGEP induced by paracetamol (acetaminophen).

CASE REPORTS

Case 1 A 33-year-old man was admitted in March 1994 with a generalized pustular eruption. He described a history of three acute episodes of pustulosis, which had resolved spontaneously within 3 weeks. The eruption began 2 days before admission, with erythema on the face and the trunk, and became disseminated in 24 h with a temperature of 39°C. He was first unsuccessfully treated at home with terfenadine and triamcinolone. On the third day, he presented with several hundred small pustules arising on widespread erythema on the flank, axillae and groin (Fig. 1). There was no evidence of mucous membrane involvement, lymphadenopathy or hepatosplenomegaly. Laboratory examination showed hyperleukocytosis, with 28.8 g/l white blood cells with 26 g/l neutrophils and no eosinophilia. C-reactive protein was elevated (266 mg/l ) and the erythrocyte sedi- mentation rate was 40 mm in the first hour. Mycology, bacteriology and virology cultures from pustules were negative and blood cultures were sterile. Cutaneous biopsy showed a subcorneal pustule. PAS staining did not reveal any pathogens. The eruption had begun 48 h after oral ingestion of 3 tablets of paracetamol (500 mg each) in one day for sinusitis. He had not ingested any other drug in the previous month. Paracetamol was stopped and there was spontaneous resolution of fever and pustules Fig. 1. Case 1 on the third day with small pustules and erythema. in 6 days, with superficial desquamation. Patch tests were performed with paracetamol (diluted 5% and 20% in saline and petrolatum) 3 weeks after subsidence of skin lesion. They non-follicular pustules. The confluence of pustules led to superficial were negative after 48 h. desquamation. No mucous membrane lesion was present and there was no fever or lymphadenopathy. Case 2 Skin biopsy demonstrated subcorneal pustules; there were slight An 83-year-old man was admitted in May 1996 with a disseminated spongiosis, papillary oedema and a perivascular infiltrate. Staining erythematous rash, which had occurred 2 days after hip replacement. with PAS did not reveal any pathogens. Within 48 h the erythematous skin was covered by hundreds of small Laboratory examination showed hyperleukocytosis, with 12.1 g/l

Acta Derm Venereol (Stockh) 78 Letters to the Editor 223 white blood cells, 10.7 g/l neutrophils and no eosinophilia. The suspected drug. Vaillant et al. (5) considered positive patch erythrocyte sedimentation rate was 100 mm in the first hour; C-reactive testing as a rechallenge test when dissemination of an eruption protein was elevated at 271 mg/l. Liver function tests showed anicteric similar to the original eruption occurred. When patch testing cholestasia (total bilirubinemia 59 mmol/l, conjugated bilirubinemia is positive without dissemination of pustules around the area 13 mmol/l, glutamyl transpeptidase 303 IU/l, alkaline phosphatase 224 tested we consider this as an argument in favour of the IU/l ), and abdominal echography was normal. He had acute renal failure (creatinemia 134 mmol/l, uremia 12.9 mmol/l ). The drugs intro- causative role of the suspected drug. duced within 2 days before the rash began were fentanyl, vecuronium A negative test does not exclude the responsibility of the bromide, cloxacillin, gentamicin, a drug combination containing par- drug, since patch testing is not a rechallenge and since penetra- acetamol, belladonna, opium and anhydrous caffeine (LamalineB) tion of the drug may be insufficient with a non-standardized and a drug combination containing paracetamol and codeine phos- patch test. phate (Efferalgan codeineB). No other drug had been ingested for 1 month before. All these drugs were stopped and the eruption resolved within 5 days, with widespread desquamation. Abnormal laboratory REFERENCES findings also disappeared in 5 days. 1. Beylot C, Bioulac P, Doutre MS. Pustulose exanthe´matique aigue Fifteen days after subsidence of skin lesion all the patch tests with ge´ne´ralise´e. A propos de 4 cas. Ann Dermatol Venereol 1980; % % paracetamol (diluted 5 and 20 in saline and petrolatum) and 107: 37–48. medication containing paracetamol were positive. They produced a 2. Roujeau JC, Bioulac-Sage P, Bourseau C, Guillaume JC, Bernard pustular eruption on an erythematous base. The histologic features of P, Lok C, et al. Acute generalized exanthematous pustulosis. the test with paracetamol crushed in petrolatum were subcorneal Analysis of 63 cases. Arch Dermatol 1991; 127: 1333–1338. pustules similar to the original lesion. Patch tests with other drugs 3. De Conynck AL, Van Strubarq AS, Pipeleers-Marichal MA, were negative. Patch testing with paracetamol was negative in 10 Huyghens LP, Suys ET, Roseeuw DI. Acute generalized exan- controls. thematous pustulosis induced by paracetamol. Dermatology 1996; Involuntary rechallenge with intravenous proparacetamol as single 193: 338–341. drug used was responsible for recurrence of AGEP 1 year later. 4. Mensig H. Akute generalisierte exanthematiche Pustulose (AGEP). H+G, Z Hautkr 1993; 68: 234–237. DISCUSSION 5. Vaillant L, Machet L, Lorette G. Les tests e´picutane´s ont-ils valeur d’un test de re´introduction en dermatologie? Therapie 1993; 48: The 2 patients presented AGEP induced by paracetamol. In 157–161. fact, these 2 cases fulfilled the criteria of AGEP except for the ff fever in the second case. The other criteria, i.e. acute di use Accepted September 24, 1997. erythema covered by amicrobial pustules, subcorneal pustule on histopathological examination and hyperleukocytosis, were F. Leger1, L. Machet1, V. Jan1, M. C. Machet2, G. Lorette1 and present. Resolution was rapid, in less than 7 days, as is usually L. Vaillant1 observed. Departments of 1Dermatology and 2Pathology, CHU Trousseau, Patch testing allows confirmation of the responsibility of a F-37044 Tours Cedex, France.

Acta Derm Venereol (Stockh) 78 224 Letters to the Editor

Acute Generalized Exanthematous Pustulosis Induced by Amoxapine

Sir, trunk, she had rapidly developed a generalized eruption accompanied Toxidermia is a well-known complication of antidepressor by fever. On examination there were symmetrically distributed pustules therapy, often related with photosensitization. We report a which were more numerous on the dorsa of the hands and on the case of amoxapine-related acute generalized exanthematous thighs (Fig. 1), but which were also present to a lesser extent on the trunk and arms. The pustules were pinhead size, and surrounded by pustulosis (AGEP) which regressed at drug withdrawal. a narrow inflammatory rim with an exfoliative pattern. Bacteriological culture of the pustules did not produce any growth of organisms. CASE REPORT Histologic features were typical of AGEP (Fig. 2). Laboratory analysis also showed a high granulocyte count (18×109/L), erythrocyte sedi- A 55-year-old woman was hospitalized with a generalized cutaneous mentation rate was 80 in the first hour. The skin eruption completely eruption. She had no personal or family history of psoriasis. Twenty disappeared within 1 week of stopping amoxapine and there was no days before her admission, oral therapy with 100 mg/day of amoxapine relapse in the following 6 months. had been started for depression. Ten days after initiation of amoxapine, the patient came for a consultation complaining of an erythema of the DISCUSSION In this case, the diagnosis of AGEP has a solid clinical and histologic basis. The involvement of amoxapine is also prob- able because it was the only drug administered before the onset of AGEP and because the skin completely cleared with no relapse after discontinuation of the therapy. To our knowledge, this case is the second report of AGEP induced by amoxapine (heterocyclic antidepressant from dibenzo- oxazepines family) (1). The most frequent causative factor involved in AGEP is reaction to drugs, mainly antibiotics (2). Although two cases of toxic epidermal necrolysis induced by amoxapine have been reported, cutaneous adverse reactions are more often photosensitization, urticaria, exanthema, eryth- ema multiforme and Stevens-Johnson syndrome, cutaneous vasculitis (3, 4). Cross-reactions between different families of antidepressants are always possible but, if necessary, it is preferable to use another family under cutaneous attention (4). Fig. 1. Non-follicular pustules on the thighs. REFERENCES

1. Larbre B, Kanitakis J, Savy C, Besnard V, Faure M, Claudy A. Acute exanthematous pustulosis during treatment with amox- apine. Ann Derm Venereol 1994; 121: 40–41. 2. Roujeau JC, Bioulac-Sage P, Bourseau C, Guillaume JC, Bernard P, Lok C. Acute generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol 1991; 127: 1333–1338. 3. Camisa C, Grines C. Amoxapine: a cause of toxic epidermal necrolysis. Arch Dermatol 1983; 119: 709–710. 4. Warnock JK, Knesevich JW. Adverse cutaneous reactions to antidepressants. Am J Psychiatr 1988; 145: 425–430.

Accepted December 8, 1997.

F. Loche, C. Durieu and J. Bazex Fig. 2. Aseptic multifocal spongiform pustule in the epidermis of the Department of Dermatology, Purpan hospital, place du, Docteur patient (haematoxylin-eosin, ×25). Baylac, F-31059 Toulouse cedex, France

Acta Derm Venereol (Stockh) 78 Letters to the Editor 225

Pitted Keratolysis: A Discussion of Two Cases in Non-weight-bearing Areas

Sir, Case 2 Pitted keratolysis (PK) is a superficial infection of the horny An otherwise healthy 20-year-old Japanese woman had a 3-month layer by gram-positive organisms, most frequently occurring history of malodorous slimy lesions on both feet in association with in tropical countries (1). The skin lesion is characterized by hyperhidrosis. The typical keratolytic lesions developed on part of the defects of the horny layer, consisting of small crateriform pits weight-bearing areas of the soles, including the ball, the heel and the toes. The non-weight-bearing areas, such as the instep and the dorsal which sometimes coalesce to form a larger depression (1–3). aspect of the toes of her right foot, were also affected in this case The most common sites for PK are the pressure-bearing areas (Fig. 2). The lesions had spread from the inner side of the right foot of the sole, such as the ventral aspect of the toe, the ball of and presented themselves as ringed keratolysis associated with a the foot and the heel, as described previously (1, 2). To the scaling collarette. She was an office worker and field-athlete. best of our knowledge, there has been no report concerning lesions on the non-weight-bearing areas of the foot among Laboratory examination people who are not in the habit of bare-foot walking. Lesions Biopsy specimens were taken from both cases using the shaving on these sites are considered to be very uncommon. We report technique, as described previously (2), and stained with haematoxylin 2 cases with PK lesions on the arch and the instep. and eosin (HE), gram, Schiff ’s periodic acid (PAS) and methenamine silver. Gram-positive coccoid and filamentous organisms were detected in the pit of the horny layer. They were also stained positively with haematoxylin, PAS and methenamine silver. Potassium hydrochloride CASE REPORTS preparations were negative for fungi in both cases. Case 1 A 12-year-old Japanese girl came to us with a 2-month history of plantar lesions on both feet, which had developed largely on the DISCUSSION arches (Fig. 1) and partly on the toes. Small crateriform pits, the characteristic and diagnostic features of PK, had coalesced to form a Pitted keratolysis was first described by Castellani as a disease larger defect of the horny layer on both sites. Soreness, unpleasant affecting bare-footed people during the rainy season in Sri smell and hyperhidrosis were associated with this condition. She had Lanka (4), and there have been many reports from other no systemic symptoms and was otherwise healthy. She had been tropical and subtropical countries (1, 5, 6). Crateriform playing volleyball for a month before the onset. defects, coalescing to form a larger plaque, are characteristic features of this disease. Hyperhidrosis is the most frequently observed symptom. Unpleasant smell and sliminess are also distinctive manifestations (2). Gram-positive organisms with coccoid and filamentous forms are usually well-demonstrated during histological examination (1).

Fig. 1. Plantar lesions of Case 1. The non-weight-bearing areas were Fig. 2. Lesions of Case 2. Non-weight-bearing areas of the foot lesion: involved. Small pits were distributed around a large depression on the instep and the dorsal aspect of the toes, presenting as ‘‘ringed the arch. keratolysis.’’

Acta Derm Venereol (Stockh) 78 226 Letters to the Editor

The weight-bearing areas are most commonly infected by 2. Takama H, Tamada Y, Yano K, Nitta Y, Ikeya T. Pitted the organisms (1). In 1931 Acton & McGuire (5) showed five keratolysis: clinical manifestations in 53 cases. Br J Dermatol 1997; clinical forms of PK among bare-footed people in Bengal, 137: 282–285. including pitted, keratolytic, fissured, paronychial and cracked 3. Takama H, Tamada Y, Ikeya T. Clinical statistics and meteorological aspects in pitted keratolysis. Jpn J Dermatol 1997; types. Besides these major types, they referred to another type 107: 1373–1380. of lesion, in which the non-weight-bearing areas, the arch and 4. Castellani A. On some subjects of tropical medicine which require the instep, were involved. There has been no other report further investigation. J Ceylon Branch Br Med Assoc 1910; 7: 1–10. concerning these forms of the disease. The instep lesion of 5. Acton HW, McGuire C. Actinomycotic lesions of the skin of the Case 2 had a characteristic clinical appearance, consisting of hands and feet, due to actinomyces keratolytica. Indian Med Gaz ‘‘ringed keratolysis’’ with a delicate collarette of scale at the 1931; 66: 65–70. periphery (6, 7). 6. Emmerson RW, Jones EW. Ringed keratolysis of the palms. Trans It is still unknown why PK lesions develop on St. Johns Hosp Dermatol Soc 1967; 53: 165–167. weight-bearing areas of the foot much more frequently than 7. Zaias N. Pitted and ringed keratolysis: a review and update. J Am on non-weight-bearing areas. It may be suggested that the Acad Dermatol 1982; 7: 787–791. non-weight-bearing areas are likely to be infected with the organisms following infection of the weight-bearing areas. Accepted October 23, 1997.

Hiromichi Takama1,2, Yasuhiko Tamada1, Kikuka Yokochi1 and 1 REFERENCES Toshihiko Ikeya 1Department of Dermatology, Aichi Medical University, 21 Yasago- 1. Zaias N, Taplin D, Rebell G. Pitted keratolysis. Arch Dermatol Karimata, Nagakute, Aichi 480-1103, and 2Takama Dermatology 1965; 92: 151–154. Clinic, Kasugai, Aichi 486-0844, Japan.

Somatostatin and Psoriasis

Sir, It was with great interest that I read the recent article on Since keratinocytes do not show receptors for somatostatin somatostatin-immunoreactivity in dendritic cells of psoriatic (3), they are unlikely to be the target for somatostatin released skin by Talme et al. (1), who observed an increased number by epidermal Merkel cells and HLA-DR-positive dendritic of such cells in psoriatic lesions and a colocalization with cells. Somatostatin receptors can be found on T-lymphocytes HLA-DR. In addition, they found some epidermal cells posit- and monocytes. An increase of somatostatin-like immunoreac- ive for both HLA-DR and somatostatin. These cells seemed tivity in the lesional epidermis of human skin, however, may to be localized in the basal and parabasal cell layers. This is suggest a hitherto unexpected role of Merkel cells in T-cell particularly interesting since we observed an increased number regulation. of epidermal Merkel cells in psoriatic lesions vs. normal skin per cm2 of skin surface (2, 3). We also observed that immuno- REFERENCES staining with antibodies against a broader range of neuropep- tides disclosed that the Merkel cells in psoriatic skin expressed 1. Talme T, Schultzberg M, Sundqvist K-G, Marcusson JA. or co-expressed immunoreactivity for neuropeptides in a higher Colocalization of somatostatin- and HLA-DR-like immunoreactiv- percentage than in normal human skin (2) (Table I). The ity in dendritic cells of psoriatic skin. Acta Derm Venereol (Stockh) findings support an ‘‘activation’’ of epidermal Merkel cells 1997; 77: 338–342. 2. Wollina U, Karsten U. Immunohistochemical demonstration of in psoriasis. cytokeratin 19-positive basal cells in psoriatic plaques. Arch Dermatol Res 1988; 280: 257–258. 3. Wollina U, Mahrle G. Epidermal Merkel cells in psoriatic lesions Table I. Expression of neuropeptides by epidermal Merkel cells – Immunohistochemical investigations on neuropeptide expression. J Dermatol Sci 1992; 3: 145–150. 4. Reubi JC, Hunziker T. Absence of somatostatin receptors in Percentage of Merkel cells positive in psoriatic lesions. Arch Dermatol Res 1990; 282: 139–141. Neuropeptide Psoriatic skin Normal skin

Somatostatin 7.0 0.0 Accepted October 13, 1997 Synaptophysin 21.7 0.0 Pancreatic polypeptide 14.8 0.0 U. Wollina Chromogranin A <3.0 <3.0 Department of Dermatology, Friedrich-Schiller-University of Jena, Erfurter Strasse 35, D-07740 Jena, Germany.

Acta Derm Venereol (Stockh) 78 Letters to the Editor 227

Pemphigus Foliaceus Associated with Cilazapril

Sir, Pemphigus is an autoimmune blistering disease characterized by circulating autoantibodies directed against the keratinocyte cell surface. However, there are numerous reports of induced pemphigus attributable to the angiotensin-converting enzyme (ACE) inhibitors captopril, enalapril and ramipril (1, 2). We report a case of pemphigus after cilazapril, a new non-thiol- containing ACE inhibitor.

CASE REPORT

A 69-year-old white woman with hypertension who had been taking cilazapril for 3 months developed numerous erythematous, crusted plaques on the scalp which spread to her back (Fig. 1), chest and Fig 2. Structure of cilazapril. Amide group indicated. face. Examination revealed several crusted plaques together with healed lesions. The Nikolsky’s sign was positive. There was neither immunofluorescence was negative to antibodies to epidermal intercellu- oral or genital involvement. A skin biopsy specimen showed acantho- lar substance and antibasement membrane. lysis with subcorneal and intramalpighian clefts (several levels of The cilazapril was discontinued but there was no spontaneous cleavage). Direct immunofluorescence of perilesional skin revealed remission of the skin lesions, so she started treatment with oral IgG and C3 deposition in the intercellular keratinocyte spaces. Indirect prednisone (120 mg/day). Presently, she has a good evolution and lesions have totally remitted with the sole administration of azathio- prine 100 mg/day.

COMMENT Drugs containing an amide group have been associated with pemphigus (4). Cilazapril has an amide group like other ACE inhibitors, and it seems likely it is responsible for inducing the pemphigus foliaceus. The 3-month delay between starting the drug, the onset of eruption, the good evolution, and the presence of several levels of cleavage in the epidermis (3) make the diagnosis of induced pemphigus most probable, which is consistent with previous reports of induced pemphigus (2).

REFERENCES

1. Kuechle MK, Hutton KP, Muller SA. Angiotensin-converting enzyme inhibitor-induced pemphigus: three case reports and literat- ure review. Mayo Clin Proc 1994; 69: 1166–1171. 2. Vignes S, Paul C, Flageul B, Dubertret L. Ramipril-induced superficial pemphigus. Br J Dermatol 1996; 135: 657–658. 3. Pen˜as PF, Jones-Caballero M. Pe´nfigo inducido por fa´rmacos. Piel 1996; 11: 297–304. 4. Wolf R, Brenner S. An active amide group in the molecule of drugs that induce pemphigus: a casual or causal relationship? Dermatology 1994; 189: 1–4.

Accepted October 27, 1997

E. Buzo´n, A. M. Pe´rez-Bernal, F. de la Pen˜a, J. J. Rı´os and F. Camacho Department of Dermatology, Hospital Universitario Virgen Fig. 1. Crusted plaques together with healed lesions on the back. Macarena, Avda Dr. Fedriani s/n . E-41007 Sevilla, Spain.

Acta Derm Venereol (Stockh) 78 228 Letters to the Editor

Eruptive Multiple Keratoacanthomas of the Extremities

Sir, lymphocytes showed a decrease of the CD19+ B cells (160 Multiple keratoacanthomas represent a heterogeneous group cells/ml, expected range 500–1500) and of the CD4+ cells of uncommon, inherited or acquired disorders in which kera- (1378 cells/ml, expected range 1700–2800) resulting in a toacanthomas, varying in number and size, develop in a decreased CD4/CD8 ratio (1.4, expected range 1.5–2.9). White localized or generalized pattern, sometimes involving the blood cell count was 6000 cells/ml, 52.2% of which were mucosae, and are occasionally associated with malignancy or lymphocytes. immunosuppression (1). Two punch biopsies from forearm lesions were performed. Eruptive keratoacanthoma is a rare variant of multiple Examination by light microscope of specimens stained with keratoacanthomas. First described by Grzybowski in 1950 (2), haematoxylin-eosin disclosed findings consistent with kera- it is characterized by the synchronous appearance of numerous toacanthoma’s diagnosis, i.e. irregular epidermal proliferations tiny keratoacanthomas (3). We present a case of multiple forming keratin-filled invaginations or entrapping keratin keratoacanthomas of the eruptive type, with unusual clinical masses and descending into the dermis. Hyperkeratosis and presentation, successfully treated with low doses of oral acanthosis were observed in the surrounding epidermis isotretinoin. (Fig. 2). Eosinophils were found among the chronic inflam- A 67-year-old female was referred to the outpatient clinic matory infiltrate. It is noteworthy that the lesion recurred at of our hospital with a 3-year history of a papular eruption on a biopsy site ( Koebnerization). the extremities. There was no family history of a similar The patient received isotretinoin 0.75 mg/kg daily, per os, condition, no history of exposure to tar, but she had been for 3 months. A significant clinical improvement was noted excessively sun-exposed during recreational activities. She on 1- and 3-month follow-up, without side effects. Most of reported hypercholesterolaemia, mild hypertension and the lesions regressed and no new lesions appeared. osteoarthritis. The appearance of the lesions in our elderly patient is an Lesions initially appeared on the forearms of the patient unusual clinical presentation of eruptive keratoacanthoma, during one summer, resolved without scarring during the with fewer and larger non-coalescing lesions, seen only on the following winter and reappeared in crops the next summer. extremities. Our case shares some features in common with This time they were located not just on the forearms, but also the patient reported by Green et al. (4), in which keratoacan- on the dorsal aspect of the hands, the anterior aspect of the thomas were localized on the upper extremities of an elderly, shins and the popliteal region as well (Fig. 1a, b). A mild heavily sun-exposed individual. pruritus was present. She had received long-term treatment Classification of multiple keratoacanthomas is difficult and with topical steroids, without clinical improvement, that is still a subject of controversy. Although there is a considerable resulted in Bateman’s purpura. However, some of the lesions degree of overlap and several unclassified cases, some morpho- regressed spontaneously after several months. logic or syndromic types have been identified (1). Physical examination revealed numerous, firm, discrete, A wide range of internal malignancies has been reported in flesh-coloured to erythematous papules, 3–7 mm in diameter, association with multiple keratoacanthomas (5). It is not clear symmetrically located on the forearms, hands and legs. Head, whether this coexistence is coincidental or represents a true neck and trunk were unaffected. Some of the papules had a association. Therefore, detailed evaluation of every patient horny plug in the centre. Lesions showed no tendency to with multiple keratoacanthomas for malignancy is warranted. coalesce. Mucous membranes were normal. Multiple keratoacanthomas are refractory to treatment. Laboratory investigation, including routine haematological Limited in number, lesions can be treated with electrodessic- and chemistry analysis, chest X-rays and CT-scan of the ation and curettage, cryotherapy, radiotherapy or intralesional abdomen, was within normal limits. Evaluation of peripheral administration of corticosteroids, bleomycin, fluoracil or podo-

Fig. 1. Several follicular papules of the forearms and hands, and of the poplietal region, some with a central horny plug. Bateman’s purpura can also be observed.

Acta Derm Venereol (Stockh) 78 Letters to the Editor 229

Fig. 2. Histology of a lesion exhibiting typical features of keratoacanthoma. Irregular epidermal hyperplasia forming keratin-filled invaginations. Hyperkeratosis with parakeratosis and orthokeratosis. Acanthosis of the epidermis. phylin (6). Systemic therapy with isotretinoin or etretinate has 6. Hendricks WM. Sudden appearance of multiple keratoacanthomas shown good results, although some of the smaller lesions may three weeks after thermal burns. Cutis 1991; 47: 410–412. remain unaffected (7, 8). Oral isotretinoin for 3 months has 7. Street ML, White JW, Gibson LE. Multiple keratoacanthomas been particularly effective in our patient. Methotrexate and, treated with oral retinoids. J Am Acad Dermatol 1990; 23: 862–866. recently, cyclophosphamide have also been employed with 8. Blitstein-Willinger E, Haas N, Nurnberger F, Stuttgen G. encouraging results and may be used as alternative therapeutic Immunological findings during treatment of multiple keratoacan- modalities in cases resistant to usual treatment (9, 10). thoma with etretinate. Br J Dermatol 1986; 114: 109–116. 9. Kestel JL Jr, Blair DS. Keratoacanthoma treated with methotrex- REFERENCES ate. Arch Dermatol 1973; 108: 723–724. 10. Carter Grine R, Hendrix JD, Greer KE. Generalized eruptive 1. Schwartz RA. Keratoacanthoma. J Am Acad Dermatol 1994; keratoacanthoma of Grzybowski: response to cyclophosphamide. 30: 1–19. J Am Acad Dermatol 1997; 36: 786–787. 2. Grzybowski M. A case of peculiar generalized epithelial tumours of the skin. Br J Dermatol Syph 1950; 62: 310–313. 3. Jaber PW, Cooper PH, Greer KE. Generalized eruptive kera- Accepted October 30, 1997. toacanthoma of Grzybowski. J Am Acad Dermatol 1993; 29: 299–304. N. G. Stavrianeas, A. C. Katoulis, N. P. Stratigeas, O. Neofotistou, 4. Green WS, Underwood LJ, Green R. Multiple keratoacanthomas E. Koumantaki-Mathioudaki and A. G. Vareltzidis on upper extremities. Arch Dermatol 1977; 113: 512–513. Department of Dermatology and Venereology, National University of 5. Snider LB, Benjamin DR. Eruptive keratoacanthoma with an Athens, School of Medicine, ‘‘A.Sygros’’ Hospital, 5, Dragoumi street, internal malignant neoplasm. Arch Dermatol 1981; 117: 788–790. GR-16121 Athens, Greece.

Acta Derm Venereol (Stockh) 78 230 Letters to the Editor

Scleroderma Renal Crisis in Association with Essential Oils

Sir, atic factor, and cold agglutinins. FANA was ++++ with nucleolar Systemic sclerosis, a connective tissue disease characterized by pattern. Capillaroscopy of the nailfolds showed great ectasies of the sclerotic skin changes and visceral organ involvement, results capillaries at the nail borders with a few haemorrhages. Double from disturbance during collagen formation and from intimal contrast esophageal X-rays revealed esophageal dysmotility. Scleroderma renal crisis was diagnosed, and treatment with captopril damage of small- and medium-sized arteries. Renal involve- ff % was added. Blood pressure gradually returned to near normal values, ment is common, a ecting some 45 of patients, and is and the ascites and pleural effusion resolved. Renal function remained associated with the highest mortality (1). The cause of the seriously disturbed, but the haemolysis stopped. The patient was disease remains unknown. discharged from hospital with controlled blood pressure and in good Scleroderma renal crisis in a patient with systemic sclerosis general condition. manifests as acute onset of malignant hypertension and pro- gressive renal failure. Its incidence in scleroderma patients is COMMENT between 10 and 25%, and if untreated leads to end-stage renal disease and death (2). About 3% of patients with scleroderma The patient’s occupation as a beautician and aromatherapist renal crisis show only limited cutaneous involvement (3). signalled a possible cause of her condition, since she had been Occupational and environmental hazards have been associ- using essential oils daily for 8 years. Essential oils are mixtures ated with the development of scleroderma or scleroderma-like of terpens, which are distilled, expressed or extracted from seeds, disease, including exposure to silica dust in miners, to epoxy bark, roots, leaves, flowers and fruit (7, 8). They are widely resins, organic solvents, trichloroethylene, polyvinylchloride, used in cosmetics as perfumes, in mouth rinses and toothpastes, or after ingestion of toxic rapeseed oil (4–6). We report here as preservatives in food (because of their anti-bacterial effect), a case of scleroderma renal crisis sine scleroderma which might and as flavourings. They are also widely used in aromatherapy be related to the patient’s occupation as an aromatherapist, as part of the pharmacopia of alternative medicine. Those used in which she is constantly exposed to essential oils through in aromatherapy are inhaled, ingested or massaged. her skin and nasal mucosa. The extensive exposure of the patient to essential oils raises the question of a possible etiologic connection between the oils CASE REPORT and the development of her scleroderma renal crisis, although the pathogenesis is not clear. It is noteworthy that although the A 60-year-old woman was admitted to the emergency room with patient’s hands and lungs were exposed daily, they showed no headache, vomiting and blood pressure of 270/130 mmHg following clinical signs of fibrosis – indicating a probable systemic rather 2 weeks of headaches and a first-time experience with high blood than local effect of the essential oils on the body. pressure. During the week preceding hospitalization she developed cold and swollen fingers, two of them turning bluish in colour. She had a history of peptic disease and osteoporosis, for which she took REFERENCES replacement therapy for 1 year. She had no history of hypertension. 1. Silver RM. Clinical aspects of systemic sclerosis (scleroderma). Physical examination on admission revealed high blood pressure Ann Rheum Dis 1991; 50 Suppl 4: 854–861. (180/90 mmHg after administration of nifedipine in the emergency 2. Gonzalez EA, Schmulbach E, Bashani B. Scleroderma renal crisis room), no papillodema, multiple telangiectasiae on the chest, a short with minimal skin involvement and no serologic evidence of systolic murmur over the apex of the heart and left sternal border, systemic sclerosis. Am J Kidney Dis 1994; 23: 317–319. and palpable peripheral pulses. The remainder of the physical examina- 3. Helfrich DJ, Banner B, Steen VD, Medsger TA. Normotensive tion, including neurological examination, chest X-ray and ECG, was renal failure in systemic sclerosis. Arthritis Rheum 1989; 32: normal. High resolution computer tomography of the lung, echocardi- 1128–1134. ography and right heart catheterization were not performed. Laboratory investigations showed Hb 8.8 gr%, mCV 95, MCH 32, 4. Silman AJ, Newman J. Genetic and environmental factors in scleroderma. Curr Opin Rheumatol 1994; 6: 607–611. BUN 47, creatinine 2.1, and LDH 1210. This contrasted with Hb 11.9 gr% and creatinine 1.1 one month earlier. Liver and muscle enzymes 5. Czirjak L, Danko K, Schlammadinger J, Suranyi P, Tamasi L, were normal. At this stage, haemolytic-uraemic syndrome due to Szeged GY. Progressive systemic sclerosis occurring in patients hormonal therapy was suspected. exposed to chemicals. Int J Dermatol 1987; 26: 374–378. Treatment with verapamil and nifedipine was administered with fresh 6. Rodnan GP, Benedek TG, Meckger TA, Jr, Cammarata RJ. The frozen plasma, but haemoglobin levels and renal function continued to association of progressive systemic sclerosis (scleroderma) with coal deteriorate and blood pressure remained uncontrollable. After several miners’ pneumoconiosis and other forms of silocosis. Ann Intern days the patient developed retinal bleeding, jugular congestion, hepato- Med 1967; 66: 323–334. megaly, ascites, and bilateral pleural effusion without lung parenchyma 7. Mahmud ALE. Antifungal action and antiaflatoxigenic properties involvement. She also complained of diffuse arthralgia. of some essential oil constituents (Letter). App Microbiol 1994; Following the development of a murmur over the left renal artery, 19: 110–113. subsequent investigations were focused on the patient’s kidneys. 8. Viollon C, Chaumont JP. Antifungal properties of essential oils Intravenous pyelography showed normal to small kidneys, and bilat- and their main components upon cryptococcus neoformans. eral decreased perfusion with no drainage problems. Abdominal Mycopathologia 1994; 128: 151–153. ultrasound revealed kidneys of normal size and shape, with no evidence of obstruction. Kidney biopsy was not performed. Accepted November 10, 1997. Based on the combination of telangiectasiae, arthralgia, malignant blood pressure, and the sudden onset of Raynaud’s phenomenon, Edith Orion1 and Sarah Brenner2 scleroderma renal crisis was suspected. Further investigations revealed Department of Dermatology, Tel Aviv Sourasky Medical Center and that C3,C4, anti-DS DNA, anti-RNP and anti-Sm were normal, and Sackler Faculty of Medicine, Tel Aviv University, Weizman Street, results were negative for anti-SCL70, anti-Ro, anti-La, ANCA, rheum- Tel Aviv 64239, Israel.

Acta Derm Venereol (Stockh) 78 Letters to the Editor 231

Chronic Cutaneous Leishmaniasis Mimicking Sebopsoriasis

Sir, Histological examination of the affected skin showed a gran- A 68-year-old man, on pharmacological substitution therapy uloma consisting of lymphocytes, plasmacytes and, mostly, for 10 years for the removal of a large pituitary adenoma with histiocytes. The routine blood work-up and the lymphocytic asymmetric expansion above the cella turcica, presented with subpopulation assays resulted as normal. chronic dermatitis of the scalp and face with onset referred 1 The patient was in poor physical condition and was therefore year earlier. Dermatologists had previously diagnosed it vari- treated intra-lesionally only, with a weekly administration of ously as psoriasis, seborrhoeic dermatitis and sebopsoriasis, 1–1.5 ml megumine antemoniate distributed in the areas clinic- but the patient claimed no improvement from the various ally affected. After six sessions of treatment the patient was topical and systemic therapies scrupulously followed. clinically healed. During examination, the patient presented diffused scalp After 2 years of total well-being, he returned to our observa- erythema and sparse whitish squamous crusts, especially on tion, presenting a clinical symptomatology similar to the one the sides. The right ear lobe appeared congested, desquamating already described, but less severe. Histological slides, prepared and sore, with serous secretion from the retroauricular groove. with the same technique as previously, revealed the presence The face was erythematous-edematous, and the skin of the of parasites outside the macrophages. A subsequent eight- nose was desquamating. A thin, irregular squamous crust session cycle of meglumine antimoniate infiltration therapy adhering to the underlying tissue was observed on the left ala resulted in a complete clinical resolution of the lesions. In the nasi (Fig. 1). Its removal uncovered a serous-producing sur- last 6 months, the patient has not had any relapses, but has face, while thin cone-like extensions emerged from the inferior developed some typical nummular psoriatic lesions on the surface of the squamous crust. Tiny pieces of tissue were taken elbows and lower limbs presently treated with topical therapy from the borders of the oozing lesion, and May-Grumwald and heliotherapy. coloured slides were prepared. At the same time, a 3 mm It is well known that focal cutaneous leishmaniasis may diameter tissue sample was punch-removed from the affected manifest itself with different clinical expressions from the right mastoidal area. classical ‘‘oriental sore’’ (1). Recently, some authors have Microscopic examination of the slides revealed the presence emphasized the rise in sporotrichoid forms with hard, mobile of numerous leishmania inside and mostly outside the macro- subcutaneous nodules which appear about a month later than phages. For this reason, no culture testing was performed. the primary lesion as an indication of parasitic diffusion along the lymphatics (2). We believe that our patient can be defined as being affected by ‘‘non-healing’’ (chronic) cutaneous leishmaniasis mimicking sebopsoriasis, a term used to define the presence of well- demarcated scalp plaques with clinical and histopathological characteristics found across psoriasis and seborrhoeic derma- titis. We cannot exclude that the clinical aspect is influenced by an isomorphic type reaction in a patient predisposed to psoriasis. A case of visceral leishmaniasis presenting as a psorasiform eruption in a young male patient affected by AIDS has been reported recently; however, neither the lymphocytic subpopulation assay nor HIV investigation was abnormal in our patient (3).

REFERENCES

1. Kubba R, Al-Gindan Y, El-Hassan AM, Omer AHS. Clinical diagnosis of cutaneous leishmaniasis (oriental sore). J Am Acad Dermatol 1987; 16: 1183–1189. 2. Kibbi AG, Hassouna L, Rubeiz NG. Cutaneous leishmaniasis: an old disease with a new face. Acta Derm Venereol (Stockh) 1996; 76: 171–172. 3. Rubio FA, Robayna G, Herranz P, Torres E, Pena JM, Contreras F, et al. Leishmaniasis presenting as a psoriasiform eruption in AIDS. Br J Dermatol 1997; 136: 792–794.

Accepted November 10, 1997.

Carmelo Schepis, MD1, Maddalena Siragusa, MD1, Antonino Alberti, MD2 and Rosaria Palazzo, MD3 1Unit of Dermatology and 2Department of Pediatrics, Oasi Institute Fig. 1. Desquamating erythematous-edematous face especially on the (IRCCS), Via Conte Ruggero, 73, I-94018 Troina, and 3Unit of nose. Dermatology, Piemonte Hospital, Messina, Italy.

Acta Derm Venereol (Stockh) 78 232 Letters to the Editor

Antipruritic Effect of Oral Cyclosporin A in Essential Senile Pruritus

Sir, RESULTS Ten patients, aged between 59 and 72 years (6 women and 4 men), affected with essential pruritus were enrolled in the All 10 subjects completed the study. The status of pruritus study after verbal informed consent was obtained. At clinical was assessed and compared with the baseline using the VAS investigation all patients presented with discomforting diffuse forms filled in by patients at each visit. CyA treatment pruritus of 6 to 11 months’ duration and which frequently significantly reduced the itch intensity in all 10 patients. Four disturbed sleep at night. None of the subjects presented any patients experienced an improvement of pruritus within a few specific skin alterations and all were resistant to oral anti- days. Six others showed a decrease in itch on days 10–12. A histamines, topical and systemic corticosteroids and topical total of eight subjects were free of pruritus within the 4th emollients. week of treatment, but two subjects who had experienced consistent itch relief by the 14th day of CyA treatment had MATERIAL AND METHODS no further improvement. At the clinical follow-up no relapse were reported until 3 months after discontinuation of therapy. Study design One subject referred a mild localized pruritus 1 month after This open uncontrolled study was designed for subjects with persistent discontinuation of therapy. pruritus for a minimum of 6 months before entry into the study. None of the patients enrolled had significant adverse reac- Routine laboratory tests, IgE level, serum complement, thyroid hor- mone levels, chest X-ray, were all within normal limits and stool tions to CyA treatment. Two patients had an increase in examination for parasites was negative in all subjects. Excluding diastolic blood pressure (100 mmHg) reversible on dose adjust- criteria to enter the study were hepatic or renal affections, pre-existing ment. No laboratory test modifications occurred in any of hypertension or history of malignancies, acute infection, pathological the subjects. routine laboratory tests, drug or alcohol abuse. Any topical and The antipruritic effect of CyA needs to be clarified, including systemic medications previously prescribed for the treatment of pru- the possible hypothesized role of CyA in inhibiting certain ritus were stopped 4 weeks before the start of the study. All subjects ‘‘pruritogenic cytokines’’ or counteracting certain neuropep- were treated with Cyclosporin A (CyA) oral solution (Sandimmun tides released in situ in the skin (1, 2). Neoral, Sandoz) 5 mg/kg/day in two administrations for 8 weeks. The initial dose was maintained for 4 weeks and then gradually reduced by 0.5 mg/kg/day every week until discontinuation of the therapy. Patients were monitored at each visit for CyA side effects, in particular REFERENCES renal or liver dysfunction and changes in blood pressure. Patients ¨ ff were followed-up for 3 months after the end of treatment to evaluate 1. Wahlgren CF, Scheynius A, Ha¨germark O. Antipruritic e ect of relapse of symptoms. oral cyclosporin A in atopic dermatitis. Acta Derm Venereol (Stockh) 1990; 70: 323–329. Recording of itching intensity 2. Teofoli P, Guarcello V, Lotti T, Panconesi E. Detection of mRNA The effects were evaluated by measuring itch intensity using a visual encoding proopiomelanocortin (POMC) in the epidermoid cell line analogue scale (VAS) in which the subjects indicate the intensity of A431. Int J Immunopathol Pharmacol 1997; 10: 1, 69–71. itch on a scale ranging from no itch to maximal itch. The assessment of the itch score on the first 2 days constituted a patient’s baseline value; the itch score was recorded on days 0, 7, 14 and 28 and then every 2 weeks until discontinuation of therapy (8 weeks). Accepted November 21, 1997

1 1 1 Statistical analysis Patrizia Teofoli , MD, Ornella De Pita , MD, Alessandra Frezzolini BS and Torello Lotti2,MD Statistical analysis was performed using Student’s t-test for paired 1Department of Immunodermatology, Istituto Dermopatico data. Data were expressed as mean values±SD and a p value less dell’Immacolata (IDI), IRCCS, Via dei Monti di Creta 104, I-00167 than 0.05 was considered significant. Rome, and 2Department of Dermatology, Florence, Italy.

Acta Derm Venereol (Stockh) 78 Letters to the Editor 233

Dermatofibrosarcoma Protuberans and Basal Cell Carcinoma

Sir, We report a case of basal cell carcinoma overlying dermato- fibrosarcoma protuberans (DFSP). A multitude of epidermal changes are commonly associated with dermatofibromas and rarely with DFSP. To our knowledge, this is the first case report of such an association between basal cell carcinoma and DFSP.

CASE REPORT

A 70-year-old male presented with an asymptomatic slowly growing brownish nodular lesion involving the left ear. The patient had noticed a rapid growth of the lesion in the previous 3 months. A biopsy followed by wide excision was performed. The biopsy consisted of a piece of skin measuring 1 cm with a firm dermal lesion. Microscopic examination showed a skin with basal cell carcinoma (Fig. 1) and a cellular dermal lesion. The lesion is composed of relatively uniform spindle-shaped cells arranged in a distinctive cartwheel or storiform pattern. Mitosis was infrequent. The tumour cells were positive for vimentin and CD34 (Signet Laboratories Inc) and negative for cytokeratin epithelial membrane antigen S100, actin and desmin Fig. 1. Basal cell carcinoma overlying dermatofibrosarcoma pro- (Dako). Their appearance indicated a diagnosis of a DFSP and basal tuberans (arrow). cell carcinoma. Further resection revealed a residual cellular tumour with similar appearance involving the subcutaneous tissue. A multitude of epidermal changes are commonly associated with dermatofibromas, and very rarely with DFSP. These DISCUSSION include epidermal acanthosis, occasional pseudoepitheliomat- ous hyperplasia, hair follicle proliferation, and basal cell DFSP, first described in 1924 as ‘‘a progressive and recurrent carcinoma-like changes. A few cases of well-documented basal dermatofibroma’’, is a nodular cutaneous tumour character- cell carcinoma have been reported (3). The most likely explana- ized by a distinctive storiform growth pattern and local tion for this coexistence of DFSP and basal cell carcinoma recurrence. It is generally regarded as a neoplasm of inter- is incidental, as the latter commonly occurs on sun-exposed mediate malignancy with rare distant metastasis. DFSP typic- skin. ally arises on the trunk and proximal extremities. Head, neck and scalp lesions have also been described. It is more frequent in men, with a peak incidence during the third decade of life (1). REFERENCES The tumour usually develops as a nodular or multinodular, 1. Conelly JH, Evans HL. Dermatofibrosarcoma Protuberans: a clinic- slowly growing cutaneous mass and appears to evolve from a opathologic review with emphasis on fibrosarcomatous area. Am dermal fibrous plaque stage. Local recurrence after simple J Surg Pathol 1992; 16: 921–925. excision occurs in 30% of cases. Metastases usually occur after 2. McLelland J, Chu T. Dermatofibrosarcoma protuberans arising in repeated recurrence, often with fibrosarcomatous transforma- BCG vaccination scar. Arch Derm 1988; 124: 496. tion and, because of this and high recurrence rate, wide 3. Goeffe LTC, Helvig EB. Basal cell carcinomas and basal cell excision is advised. carcinoma-like changes overlying dermatofibromas. Arch Derm Histogenesis of the DFSP is controversial. Fibroblastic, 1975; 111: 589–599. histiocytic or perineural cells have been suggested as the cell of derivation. DFSP is considered to have no precipitating Accepted November 24, 1997. cause and in most case reports no comment is made of possible etiological factors. A history of prior trauma is usually men- K. Ibrahiem and J. M. Radhi tioned following BCG vaccination and ionizing radiation for Department of Pathology, Royal University Hospital, 107 Hospital basal cell carcinoma (2). Drive, Saskatoon, Saskatchewan, S7N 0W8, Canada.

Acta Derm Venereol (Stockh) 78 234 Letters to the Editor

Herpes Zoster

Sir, and gB glycoproteins leads to a non-cytolytic virus/ker- We read with interest the article entitled ‘‘Histopathological atinocyte relationship resulting in chronic infection. A similar findings, viral DNA distribution and lymphocytic immuno- type of low-productive virus/host cell relationship was also phenotypes in vesicular and papular types of herpes zoster’’, detected in keratinocytes of chronic HSV and VZV lichenoid recently published in the journal (1). We are pleased to see dermatosis (9). that the authors confirm several of our previous findings, Our results underline the importance of pushing investi- despite the fact that they do not quote these works. gations further on in vivo viral gene expression in order to In fact, in various stages of herpes zoster, including the disclose possible altered patterns that may be related to the erythematous, papular, vesicular and crusted stages, we com- pathomechanisms of atypical clinical presentations. pared by immunohistochemistry (IHC) the distribution of Herpes Simplex Virus (HSV ) I and II and Varicella Zoster REFERENCES Virus (VZV ) specific antigens (2). It was reported that HSV 1. Yi JY, Kim TY, Shim JH, Cho BK, Kim CW. Histopathological could only be detected in epithelial structures, whereas VZV findings, viral DNA distribution and lymphocytic immunopheno- was often present in both epidermal and dermal cell popula- types in vesicular and papular types of herpes zoster. Acta Derm tions, including type I dendrocytes. Such findings shed some Venereol (Stockh) 1997; 77: 194–197. light on the occurrence of vasculitis, granulomas and scars at 2. Nikkels AF, Debrus S, Sadzot-Delvaux C, Piette J, Delvenne P, the site of former acute herpes zoster (2). Rentier B, Pie´rard GE. Comparative immunohistochemical study Furthermore, we performed a study using IHC and in situ of herpes simplex and varicella-zoster infections. Virchows Arch A hybridization (ISH ), detecting the VZV envelope glycoproteins 1993; 422: 121–126. gE and gB and their corresponding nucleic acid sequences in 3. Nikkels AF, Delvenne P, Debrus S, Sadzot-Delvaux C, Piette J, Rentier B, Pie´rard GE. Distribution of varicella zoster virus gpI various stages of varicella and herpes zoster skin lesions (3). and gpII and corresponding genome sequences in the skin. J Med Comparing the VZV/host cell relationship in epidermal and Virol 1995; 40: 91–96. dermal cells we demonstrated a cell type-dependent permiss- 4. Muraki R, Iwasaki T, Sata T, Sato Y, Kurata T. Hair follicle iveness to VZV infection. The immunophenotype of the involvement in herpes zoster: pathway of viral spread from ganglia inflammatory infiltrate during herpes zoster and varicella skin to skin. Virchows Arch 1996; 428: 275–280. rash was also discussed in these papers (2, 3). 5. Nikkels AF, Debrus S, Sadzot-Delvaux C, Piette J, Rentier B, The importance of the pilosebaceous structure in the patho- Pie´rard GE. Localization of varicella-zoster virus nucleic acids and genic pathway of herpes zoster skin lesions was underlined in proteins in the human skin. Neurology 1995; 45: 47–49. 6. Weinberg JM, Mysliwiec A, Turiansky GW, Redfield R, James these studies (2, 3) and by Muraki and coworkers (4). Our WD. Viral folliculitis. Arch Dermatol 1997; 133: 983–986. observations also led to the individualization of follicular 7. Nikkels AF, Debrus S, Delvenne P, Piette J, Rentier B, Pie´rard herpes zoster, characterized by viral replication restricted to GE. Viral glycoproteins in herpes viridae granulomas. Am the follicular keratinocytes. In a conceptual point of view, J Dermatopathol 1994; 16: 588–592. follicular herpes zoster might be situated between zoster sine 8. Nikkels AF, Rentier B, Pie´rard GE. Chronic varicella zoster virus herpete and regular herpes zoster (5). Recently, this concept skin lesions in patients with human immunodeficiency virus are was beautifully illustrated in a case report (case 4) by Weinberg related to decreased expression of gE and gB. J Infect Dis 1997; and coworkers (6). 176: 261–264. 9. Nikkels AF, Sadzot-Delvaux C, Rentier B, Pie´rard-Franchimont C, Our findings also shed new light on the pathogenesis of post Pie´rard GE. Low-productive alpha-herpesviridae infection in zosterian granulomatous reactions most probably related to a chronic lichenoid dermatosis. Dermatology (in press). delayed type hypersensitivity reaction to persisting VZV envel- ope glycoproteins in the deep dermis while unrelated to the Accepted November 17, 1997. presence of viral DNA (7). More recently, an altered gene expression pattern of VZV Dr. A.F. Nikkels and Prof. G.E. Pie´rard was shown during chronic hyperkeratotic skin infections in Department of Dermatopathology, University of Lie`ge, CHU AIDS patients (8). The astounding reduced expression of gE Sart Tilman, B-4000 Lie`ge, Belgium.

Response to the Letter by Nikkels & Pie´rard

It is my clinical opinion that herpes zoster in patients with virus in the tissue. Nikkels & Pie´rard indicated that there persistent papules without vesicular change are found fairly were some rare atypical clinical presentations in herpes zoster, frequently in Korea. In our study to elucidate differences and in previous studies the varicella zoster virus could have between the classical vesicular and papular types of herpes involved a pilosebaceous unit. But no mention was made zoster, it was found that the appearance of clinical types of of a precise correlation between virological and clinical herpes zoster depends on the distribution of varicella zoster findings.

Acta Derm Venereol (Stockh) 78 Letters to the Editor 235

We believe that it is necessary to distinguish between major Jong Yuk Yi clinical types (vesicular or papular types) when studying the Department of Dermatology, UiJongBu St. Mary’s Hospital, Catholic pathogenesis and clinical efficacy of therapeutic agents in University Medical College, 65–1 KumOh-dong, UiJongBu-city, KyungGi-do 480–130, Korea. herpes zoster.

Focal Dermal Hypoplasia: A Case with Minor Clinical Manifestations

Sir, up to the root of both limbs. They involved a band of skin appearing Since Goltz (1) first coined the term ‘‘focal dermal hypoplasia’’ as atrophic-hypochromic, partly reticulated areas interspersed with in the 1960s, over 200 cases have been described worldwide. reddish papules and small squamocrusts (Fig. 1). The nail plate of The syndrome may involve the skin, the skeleton, the ocular the right hallux showed a longitudinal dystrophic fissure in the middle. The lower lateral incisor teeth were cone-shaped and hypoplastic. system, or, less frequently, the renal, gastroesophageal, audit- Clinical chemistry tests (in particular total IgE, ANA, antiENA ory and nervous systems. Here we report the case of a patient antibodies, total urinary porphyrins and blood protoporphyrins) were who presented with less definite clinical features than normal. Histologic examination of a papule from an atrophic patch commonly described. revealed mild irregular acanthosis with focal parakeratosis in the central portion of the lesion. The dermis was thinned and edematous. The fat was located abnormally high (Fig. 2). CASE REPORT X-rays of the upper limbs showed small calcareous deposits and Our patient, a 5-year-old girl in good general condition, was a first radiopaque symmetrical, horizontal striae in correspondence with the child, and her mother had not had any miscarriages. Family history distal metaphyses of humerus. A phototest revealed a higher than was negative for skin diseases. In the third month of life she had normal and long-lasting UV-B skin reactivity. Eye and neurological shown mild papulocrusty lesions on the lower limbs that worsened examinations were negative. The chromosome map showed a 46 XX during the summer and became itchy. The lesions were regarded as karyotype with no structural changes of the chromosomes. atopic dermatitis. When first observed by us, the lesions were affecting the flexor surface of the right lower limb and partially of the left one, DISCUSSION Our patient failed to show the most typical features of the Goltz syndrome, i.e. extroversion of the adipose tissue and eye involvement. Histology and bone X-rays were therefore conclusive for a correct diagnosis. Histological examination revealed a typical picture, but the radiological picture of our patient lacked the classic longitudinal distribution. Normally, the striated osteopathy appears as a net of condensed striae parallel to the longitudinal axis of the bone. The striae usually originate at metaphysis level in correspondence with the osteogenesis areas, where they look thicker and then gradually thin down as they go up along the diaphysis. They are usually bilateral and symmetrical, mainly involving the long bones and the sacral bone, while the vertebrae and the iliac bones are spared (2). Cases showing a less evident

Fig. 1. Band of poikilodermatous skin with papules and squamo- Fig. 2. Dermis thinned an edematous; fat located abnormally high crusts lesions. (hematoxylin-eosin stain, 25×).

Acta Derm Venereol (Stockh) 78 236 Letters to the Editor striated osteopathy and often malformation are reported in Strie´e, symptome radiologique de l’hypoplasie dermique en aires. the literature (3). Skeletal System 1972; 15: 287–295. Focal dermal hypoplasia can present with negligible clinical 3. Boothroyd AE, Hall CM. The radiological features of Goltz syndrome: focal dermal hypoplasia. Skeletal Radiol 1988; 17: features, as described previously (4, 5). In one of the two 505–508. patients reported by Pujol the skin features were as in our 4. Pujol RM, Casanova JM, Pe´rez M, Matias-Guiu X, Planaguma` patient, but in the other the diagnosis was made only after M, De Moragas JM. Focal dermal hypoplasia (Goltz Syndrome): the women had undergone several miscarriages and had given report of two cases with minor cutaneous and extracutaneous birth to a baby girl with quite evident signs of Goltz manifestations. Pediatr Dermatol 1992; 9: 112–116. syndrome. 5. Van der Kerkhof PCM, Perret CM, Happle R. Goltz-Gorlin Syndrom ohne foikale dermale hypoplasie. Hautarzt 1988; 39: 743–745.

REFERENCES Accepted November 19, 1997

1. Goltz RW. Focal dermal hypoplasia syndrome. Arch Dermatol S. Menni, D. Boccardi and D. Imondi 1992; 128: 1108–1111. Istituto di Scienze Dermatologiche, University of Milan, I.R.C.C.S., 2. Larregue M, Maroteaux P, Michel Y, Faure´ C. L’Oste´opathie Ospedale Maggiore, Via Pace n° 9, I-20122 Milan, Italy.

Verrucous-crusted Herpes Zoster in an Immunocompetent Patient

Sir, We present a case of verrucous-crusted herpes zoster in an immunocompetent patient.

CASE REPORT

A 77-year-old woman was admitted to our Institute because of a suspected verrucous-crusted herpes zoster. The patient reported that 3 months previously she had been admitted to another hospital because of a herpes zoster of the ophthalmic branch of the left trigeminal nerve, accompanied by keratoconjunctivitis. The patient was treated with acyclovir cream (5 applications/day for 7 days), acyclovir ophthalmic ointment (5 applications/day for 7 days) and oral diclofenac (100 mg/day for 7 days). Clinical manifestations regressed after approximately 1 month. Some weeks later, however, new erythemato-vesicular lesions appeared at the same sites, suggestive of a recurrence of the previous herpes zoster, as well as ulcerative lesions in the cornea. The patient was treated with acyclovir cream, acy- Fig. 1. Verrucous-crusted herpes zoster. clovir and tobramycin ophthalmic ointments and atropine collyrium. At the time of admission to our Institute, the patient presented with far as the keratoconjunctivitis is concerned, the patient was treated a verrucous-crusted lesion which was localized to the left fronto- with atropine collyrium and acyclovir ophthalmic ointment (5 parietal region, corresponding to the previous herpes zoster. The applications/day for 10 days). Complete resolution of cutaneous lesion was constituted by a single verrucous-crusted plaque, yellowish- lesions and pain was observed within 2 weeks. No recurrences were brown in colour, with irregular and rugged surface (Fig. 1). The observed in a 12-month follow-up period. plaque was firmly adherent to the underlying surface and had a hard consistency. Partial removal of a portion of the plaque with 20% salicylic acid ointment revealed an underlying erythemato-erosive area. DISCUSSION Left eyelids were erythemato-oedematous; furthermore, purulent conjunctivitis was visible. The cornea was diffusely oedematous and At least three clinical varieties of herpes zoster have been with a wide central ulcer. The patient complained of burning and described up to now. Bullous herpes zoster is characterized pain. General physical examination did not reveal anything by blisters which may appear d’emble´e or by coalescence pathological. of multiple vesicles. Ulcerative-necrotic-gangrenous and 3 Laboratory tests showed leukocytosis (15.700 WBC/mm ) and verrucous-crusted (hyperkeratotic) herpes zoster are observed increase in ESR (60 mm at the first hour). Only anti-varicella-zoster almost exclusively in immunodepressed patients, particularly virus (VZV) IgG was positive. because of HIV infection (1–8). Cytological examination, carried out on the eroded area under the verrucous-crusted lesion, showed the presence of numerous giant and From a clinical point of view, verrucous-crusted herpes multinucleated keratinocytes. Instrumental investigations were zoster is characterized by the slow but progressive development negative. of verrucosities and crusts. Histologically, apart from the The patient was treated with intravenous acyclovir (5 mg/kg 3 times typical herpes zoster picture, the lesions present an epidermal a day for 7 days) and oral diclofenac (100 mg/day for 10 days). As and adnexal pseudo-carcinomatous hyperplasia with marked

Acta Derm Venereol (Stockh) 78 Letters to the Editor 237 hyperkeratosis (1–8). These histopathological findings suggest SC, Biron KK. Acyclovir-resistant varicella zoster virus infection that verrucous-crusted herpes zoster might represent an over- after chronic oral acyclovir therapy in patients with the acquired response to a chronic infection by VZV. The appearance of immunodeficiency syndrome (AIDS). Ann Intern Med 1990; 112: verrucous-crusted herpes zoster in an immunocompetent 187–191. 4. Hoppenjans WB, Bibler MR, Orme RL, Solinger AM. Prolonged patient is an event which, to our knowledge, has never been cutaneous herpes zoster in acquired immunodeficiency syndrome. reported in the literature. In the case we have described, the Arch Dermatol 1990; 126: 1048–1050. only hypothesis that can be put forward is that of a VZV 5. LeBoit PE, Lı´mova´ M, Yen TSB, Palefsky JM, White CR Jr, infection that became chronic-recurrent as a result of an Berger TG. Chronic verrucous varicella-zoster virus infection in incorrect therapy: in fact, the patient was previously treated patients with the acquired immunodeficiency syndrome (AIDS). only with topical acyclovir. In immunodepressed patients, the Histologic and molecular biologic findings. Am J Dermatopathol most frequent cause of verrucous-crusted herpes zoster is 1992; 14: 1–7. represented by a resistance to acyclovir (1–8). This hypothesis 6. Grossman MC, Grossman ME. Chronic hyperkeratotic herpes is not plausible in our patient, since therapy with acyclovir by zoster and human immunodeficiency virus infection. J Am Acad the intravenous route resulted in a rapid, complete and long- Dermatol 1993; 28: 306–308. 7. Vaughan Jones SA, McGibbon DH, Bradbeer CS. Chronic lasting resolution of skin lesions. verrucous varicella-zoster infection in a patient with AIDS. Clin Exp Dermatol 1994; 19: 327–329. 8. Veenstra J, Van Praag RME, Krol A, Wertheim Van Dillen PME, REFERENCES Weigel HM, Schellekens PTA, et al. Complications of varicella 1. Pahwa S, Biron K, Lim W, Swenson P, Kaplan MH, Sadick N, zoster virus reactivation in HIV-infected homosexual men. AIDS Pahwa R. Continuous varicella-zoster infection associated with 1996; 10: 393–399. acyclovir resistance in a child with AIDS. JAMA 1988; 260: 2879–2882. Accepted November 19, 1997. 2. Linnemann CC Jr, Biron KK, Hoppenjans WG, Solinger AM. Emergence of acyclovir-resistant varicella zoster virus in an AIDS S. Veraldi, L. Gnecchi and F. Zorzi patient on prolonged acyclovir therapy. AIDS 1990; 4: 577–579. Institute of Dermatological Sciences, I.R.C.C.S., University of Milan, 3. Jacobson MA, Berger TG, Fikrig S, Becherer P, Moohr JW, Stanat Via Pace 9, I-20122 Milan, Italy

Erythema Annulare Centrifugum Associated with Pregnancy

Sir, serum urea nitrogen, creatinine, liver function test, VDRL and Erythema annulare centrifugum (EAC) or gyrate erythema antinuclear antibody. starts as erythematous papules or plaques that expand in a A diagnosis of EAC was made. One week later she was hospitalized centrifugal pattern. The centre of the expanding lesion consists and delivered a healthy baby, weighing 2460 g, whose general condition was good. The improvement of the eruption was noted after delivery. of somewhat hyperpigmented skin and the edge of the lesion Within 3 days, all skin lesions had almost disappeared. No evidence has trailing scale behind the advancing border. The condition of recurrence was seen after 6 months. has been categorized into superficial and deep variants. Vesiculation is rare. The pathogenesis of EAC is not fully understood, but it is thought to represent a cutaneous hyper- DISCUSSION sensitivity to diverse causes (1, 2). Here we report a case of EAC associated with pregnancy There are several reports of EAC in association with a variety of underlying disorders, including infection (bacterium, virus, fungus or parasite), drugs (salicylates, chloroquine, hydrochlo- CASE REPORT rothiazide or penicillin), blue cheese Penicillium and neoplasm A 27-year-old Thai woman presented in the 36th week of pregnancy (1–4). Unfortunately, the evidence for these associations is with a 3-week history of skin eruptions on the abdomen and extremit- based on one or only a few patients. Two aetiologic factors, ies. The skin lesions began with asymptomatic erythematous papules including cancer and dermatophytes, are more significant in before spreading gradually to form large rings. She was otherwise well that they have been reported much more frequently. In addi- and this was her first pregnancy. She took only iron supplement tion, there is experimental evidence to suggest dermatophytes during pregnancy. as a cause of EAC (1). Transformation of a Trichophyton Cutaneous examination revealed erythematous polycyclic lesions intradermal skin test into a typical EAC was described. The with central clearing and trailing rim of fine scale on the abdomen, tumours associated with EAC may be solid or haematologic, forearms and legs (Fig. 1). benign or malignant. EAC resolved following successful treat- A potassium hydroxide examination was performed and a negative result obtained. Histology of a biopsy specimen from the edge of skin ment of the neoplasia and recurrence of EAC was noted lesion showed a dense perivascular lymphocytic infiltrate in the upper concurrently with tumour relapse (5). However, the rapid dermis. Direct immunofluorescence was negative for IgG, IgA, IgM, resolution of EAC post-delivery in our patient suggests a C1q,C3,C4 and fibrinogen. The results of the following laboratory causal relationship. To our knowledge, no case of EAC has investigations were normal: complete blood count, urinary analysis, been reported in association with pregnancy. Kelly et al. (6)

Acta Derm Venereol (Stockh) 78 238 Letters to the Editor

reported a skin lesion resembling EAC in pregnant woman, but the histology showed mild leukocytoclastic vasculitis. So the diagnosis was consistent with annular vasculitis. The exact mechanism of the association of pregnancy and EAC is unknown. However, during pregnancy, there are significant and complex physiological changes, especially pro- tein and steroid hormones. These hormones may influence the immunology and inflammatory response of the skin. Furthermore, there have been previous reports of EAC which have also described following oestrogenic compounds (3) and menstrual cycle (2). We therefore believe that EAC in our patient is causally related to pregnancy. The eruptions dramatically resolved within a few days of delivery.

REFERENCES

1. White JW Jr. Gyrate erythema. Dermatol Clin 1985; 3: 129–139. 2. Tsuji T, Kadoya A. Erythema annulare centrifugum associated with liver disease. Arch Dermatol 1986; 122: 1239–1240. 3. Goette DK, Beatrice E. Erythema annulare centrifugum caused by hydrochlorothiazide-induced interstitial nephritis. Int J Dermatol 1988; 27; 129–130. 4. Summerly R. The figurate erythemas and neoplasia. Br J Dermatol 1964; 76: 370–373. 5. Yaniv R, Spielberg O, Shapiro D, Feinstein A, Ben-Bassat I. Erythema annulare centrifugum as the presenting sign of Hodgkin’s disease. Int J Dermatol 1993; 32: 59–61. 6. Kelly RI, Cook MG, Marsden RA. Annular vasculitis associated with pregnancy. Br J Dermatol 1933; 129: 599–601.

Accepted November 26, 1997.

Charoen Choonhakarn and Pitulak Seramethakun Division of Dermatology, Department of Medicine, Faculty of Fig. 1. Erythematous polycyclic lesions with trailing scale on left Medicine, Srinagarind Hospital Medical School, Khon Kaen forearm. University, Khon Kaen 40002, Thailand.

Becker’s Naevus of the Lower Limb

Sir, ules on the lateral aspect of the right knee since 8 years. Hypertrichosis Becker’s naevus (pigmented hairy epidermal naevus) is usually was present in the central macule (Fig 1). located on the shoulder, anterior chest and scapular region. The lesion appears during adolescence and usually hyper- Case 3 trichosis develops a few years later within the pigmented area, Since 10 years of age, a 24-year-old labourer had had a large dark- with an increase in size of lesion during this period. In the brown macule with peripheral light-brown smaller macules extending from the upper border of the left iliac crest to the ankle, involving the past year we have seen five Becker’s naevi involving the lower % lateral aspect of the lower limb. There was no induration or epidermal limb, a site reported in only 3 of the patients (1). thickening. Hypertrichosis was conspicuous throughout the larger macule, mainly in the central part. CASE REPORTS Case 4 Case 1 A 26-year-old student who had noticed a gradually increasing asympto- An 18-year-old male who presented with a 4-year history of light matic brown macule extending from the lower third of his thigh to brown pigmentation with a central large macule (8×6 cm) and the calf area on the right lower limb since he was 15 years of age. He scattered smaller macules at the periphery (‘‘splash on’’ appearance) had noticed increased hair growth in the lesion for the last 5 years. on the anterolateral aspect of the left thigh. He had noticed hyper- trichosis on the pigmented area since 2 years. Case 5 A 16-year-old boy who had developed a brownish macule 10 cm in Case 2 size on the right thigh at the age of 12 years. The lesion had a typical A 15-year-old boy who had developed an asymptomatic dark brown ‘‘splash on’’ appearance in the periphery and hypertrichosis in the macule (6×5 cm) with peripheral scattered smaller light brown mac- central part.

Acta Derm Venereol (Stockh) 78 Letters to the Editor 239

basal layer pigment in all of them, while in two patients some melanophages and pigment incontinence was also seen. Complete haemogram, liver and kidney function tests, serum chemistry and routine urine analysis revealed no abnormalities.

COMMENTS The belief that these naevi involve the lower limbs uncommonly may perhaps be related to the preponderance of the blemish in males, who may be relatively less concerned about the cosmetic appearance of the lower limbs than females. Moreover, lesions of the covered areas which are completely asymptomatic may not prompt the patient to seek medical advice. The finding of these naevi on the lower limbs has prompted us to believe that the involvement of the lower limb may not be very uncommon and that the legs do not seem to be preferentially spared, as emphasized in the literature.

REFERENCE

1. Tymen R, Forestier JF, Boutet B, Colomb D. Naevus tardif de Becker: a propos d’une serie de 100 observations. Ann Dermatol Venereol 1981; 108: 41–46.

Fig. 1. Becker’s naevus on the right knee with hypertrichosis in the central macule. Accepted December 10, 1997.

There were no other naevi or cafe-au-lait spots elsewhere on the Binod K. Khaitan, Devraj Dogra, Yashpal Manchanda and Sanjay body in these patients. General physical and systemic examination Rathi revealed no abnormality except the presence of BT Hansen in one Department of Dermatology & Venereology, All India Institute of patient. Skin biopsies performed in four patients showed increased Medical Sciences, New Delhi 110029, India.

ERRATUM

Re: The paper by Biagioni PA and Lamey P-J. Acyclovir non-improved, and no relationship was observed between Cream Prevents Clinical and Thermographic Progression of treatment outcome and the time elapsed before treatment Recrudescent Herpes Labialis beyond the Prodromal Stage. began. They were 1.1±0.3, 1.0±2.5, and 1.1±0.25, respect- Acta Derm Venereol (Stockh) 1998; 78: 46–47. In the last ively, and the time before treatment values was 6.8h±6.3, paragraph before Discussion, an error has occurred. The ‘‘time 7.1h±6.4 and 7.8h±6.1, respectively. Three patients reported before treatment values’’ should read ‘‘6.8h±6.3, 7.1h±6.4 adverse effects: one reported a dry flaking area, which affected and 7.8h±6.1, respectively.’’ The correct paragraph is shown the uninvolved skin to which the cream was applied, and here. 2 patients reported a stinging sensation on applying the ‘‘No difference was observed in the Dt° C values for the cream.’’ three outcome groups, i.e. prevented, clinically improved and

Acta Derm Venereol (Stockh) 78

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