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August 2007

Late-Onset Familial Mediterranean Fever: An Atypical Presentation of Dermatologic Interest

Arch Dermatol. 2007;143(8):1073-1087. doi:10.1001/archderm.143.8.1080

Familial Mediterranean fever (FMF) is a recessively inherited autoinflammatory disease characterized by self-limited bouts of fever and polyserositis. Onset usually occurs in childhood, and reactive amyloidosis may be a life-threatening complication in adult life.1 As for other autoinflammatory syndromes, the skin is an important target tissue for inflammation. In FMF, the appearance of an erysipeloid erythema, typically monolateral and localized to the lower leg and on the dorsal surface of the foot,2 is pathognomonic in the context of systemic inflammation, including fever, serositis, and synovitis. However, the clinical significance of such a cutaneous feature is difficult to define in patients without a disease-associated history or in the absence of a positive genetic test finding.