A case of atypical scleromyxedema mimicking angioedema which responded well to acitretin treatment

A 37-year-old male patient was admitted our clinic with complaints of edema and mild pruritus on the eyelids, ears, and neck. On dermatological examination there were pale erythema and edema on eyelids and ears; also coarsening of the facial skin and grooving in the forehead lines. In his medical history, it was learned that systemic corticosteroids, antihistamines and epinephrine treatments were given with the diagnosis of urticaria, angioedema for about two months, but he did not benefit from these treatments. A diagnosis of atypical scleromyxedema without monoclonal gammopathy was done according to the clinical, histopathological and laboratory findings. Acitretin treatment with a dose of 35 mg/day was started. Marked regression in the lesions was observed two months later.

Anjiyoödemi taklit eden ve asitretin tedavisine iyi yanýt veren atipik skleromiksödemli bir olgu

Otuz yedi yaþýndaki erkek hasta göz kapaklarýnda, kulaklarýnda, boyun ön ve yan yüzleri ile ensesinde þiþlik ve hafif kaþýntý þikayeti ile polikliniðimize baþvurdu. Dermatolojik muayenesinde göz kapaklarýnda ve kulaklarýnda soluk eritem ve ödem; yüz derisinde kabalaþma ve alýn bölgesinde oluklanmalar saptandý. Hastanýn öyküsünden yaklaþýk iki aydýr ürtiker-anjioödem tanýlarý ile sistemik kortikosteroid, antihistaminik ve adrenalin tedavilerinin verildiði fakat bu tedavilerden hiç fayda görmediði öðrenildi. Klinik, histopatolojik ve laboratuar bulgularýnýn ýþýðýnda hastaya monoklonal gamopatisiz atipik skleromiksödem tanýsý konuldu. Hastaya asitretin (35 mg/gün) tedavisi baþlandý. Ýki ay sonra lezyonlarda belirgin düzelme gözlendi.