Skin and muscle involvement as presenting symptoms in four children with familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent attacks of fever and polyserositis. It is the most frequent periodic fever syndrome. In FMF, sterile peritonitis, pleuritis and arthritis are frequently seen in addition to recurrent febrile attacks. Skin and muscle involvement is less common. Here, we report four patients presented with skin lesions or myalgia. Most striking findings in those patients are the absence of other major criteria for FMF and dominancy of skin lesions or myalgia. All four patients had MEFV gene mutations on both alleles. In patients with erysipelas-like lesions or erythema nodosum along with arthritis/arthralgia or recurrent myalgia, FMF should be kept in mind.