The cutaneous manifestations in children with familial Mediterranean fever (recurrent hereditary polyserositis). A six-year study.

In a six-year study of 46 children with familial Mediterranean fever (recurrent hereditary polyserositis), 20 children (43 per cent) developed cutaneous manifestations. Ten children had 18 episodes of erysipelas-like erythema which proved to be specific for the disease. Twelve children (26 per cent) had 31 episodes of non-specific purpuric rash and six children (13 per cent) had nine episodes of Henoch-Schönlein purpura. Other manifestations included diffuse erythema of the face, and/or trunk, angioneurotic oedema, diffuse erythema of the palms and soles followed by mild desquamation of the skin, Raynaud's phenomenon and a subcutaneous nodule. The mean frequency of the cutaneous lesions was 1.6/patient/year before colchicine therapy and 0.4/patient/year after colchicine therapy (p = 0.0005). The high incidence of cutaneous manifestations and their response to colchicine strongly suggest that skin involvement is an integral part familial Mediterranean fever.