Neutrophilic lobular panniculitis as an expression of a widened spectrum of familial mediterranean fever.

Neutrophilic Lobular Panniculitis as an Expression of aWidened Spectrumof Familial Mediterranean Fever Familial Mediterranean fever (FMF) is considered to be an autosomal recessive disease, though it is controversial.1-3 The marenostrin-encoding fever gene (MEFV) is responsible for FMF. The most frequent mutation is M694V, which represents a genetic risk factor for development of amyloidosis1 and more severe disease. The classic clinical picture consists of generally short recurrent febrile episodes, serositis, and erysipelas-like erythema (ELE). Colchicine is the treatment of choice for prevention of the attacks and AA amyloidosis. With isolation of the MEFV gene in 1997, incomplete or “atypical” forms of FMF have been reported and are considered by some authors an expanded spectrum of the disease.2