Manifestations cutanées rares de la maladie périodique

Abstract

Familial mediterranean fever belongs to the periodic fever syndromes. During the attacks, fever is associated with abdominal pain, arthralgia, or both. Cutaneous involvement occurs in 7 to 46 p. 100 of cases and mainly consists in erysipelas-like erythema. We report on three patients treated for familial Mediterranean fever who developed unusual cutaneous lesions. All the patients had long past history of familial mediterranean fever without cutaneous involvement except, for the third patient who had pseudo-erysipela. The first patient had diffuse Sweet's syndrome-like lesions, the second developed long lasting panniculitis of the thigh and the third had a persistent and lichenified erysipela-like plaque. In two patients, skin histology revealed an inflammatory infiltrate with neutrophils. In all cases, an increase in the colchicine dose led to the rapid resolution of the lesions. In our 3 case reports, the lesions were particular because of their atypical clinical appearance, their long duration, and they differed from the usual pseudo-erysipela aspect. Histopathologically, the lesions were similar to pseudo-erysipela, which has led some authors to hypothesize that cutaneous lesions of familial mediterranean fever belong to neutrophilic dermatoses. This hypothesis is supported by the response to the increase in colchicine doses.