title: Skin and muscle involvement as presenting symptoms in four children with familial Mediterranean fever.
abstract: 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.
authors.affiliation: Departments of Pediatrics, School of Medicine, Dokuz Eylül University, Mithatpaşa Cad. 665/4, Küçükyali, 35280, Izmir, Turkey. s.kavukcu@deu.edu.tr
authors.forename: Salih
authors.initials: S
authors.lastname: Kavukcu
authors.forename: Mehmet
authors.initials: M
authors.lastname: Türkmen
authors.forename: Alper
authors.initials: A
authors.lastname: Soylu
authors.forename: Belde
authors.initials: B
authors.lastname: Kasap
authors.forename: Burçak
authors.initials: B
authors.lastname: Tatli Güneş
mesh_terms: D000293:Adolescent; D002648:Child; D003078:Colchicine / Q000627:therapeutic use; D004893:Erythema Nodosum / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D010505:Familial Mediterranean Fever / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D006801:Humans; D008297:Male; D018482:Muscle, Skeletal / Q000473:pathology*; D009135:Muscular Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D012074:Remission Induction; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D003078:Colchicine