title: Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever.
abstract: Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease, which is characterized by recurrent and paroxysmal fever, peritonitis, arthritis, myalgia, and skin rashes. Although various skin lesions such as "erysipelas-like erythema", urticaria, nonspecific purpura, and subcutaneous nodules have been described, cutaneous vasculitis is rare. We report a Japanese case of sporadic FMF accompanied by cutaneous arteritis at the time of febrile attacks of FMF. Gene analysis revealed M694I mutation in a single allele of the MEFV gene, and oral colchicine successfully controlled both periodic fever and subcutaneous nodules of arteritis. Cutaneous necrotizing vasculitis repeatedly emerging with febrile attacks should be included among the skin manifestations of FMF.
authors.affiliation: Department of Dermatology, Asahikawa Medical University, Asahikawa, Japan.
authors.forename: Shigetsuna
authors.initials: S
authors.lastname: Komatsu
authors.forename: Masaru
authors.initials: M
authors.lastname: Honma
authors.forename: Satomi
authors.initials: S
authors.lastname: Igawa
authors.forename: Hitomi
authors.initials: H
authors.lastname: Tsuji
authors.forename: Akemi
authors.initials: A
authors.lastname: Ishida-Yamamoto
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Migita
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Ida
authors.forename: Hajime
authors.initials: H
authors.lastname: Iizuka
mesh_terms: D000328:Adult; D001167:Arteritis / Q000188:drug therapy / Q000209:etiology*; D003078:Colchicine / Q000627:therapeutic use*; D003598:Cytoskeletal Proteins / Q000235:genetics*; D010505:Familial Mediterranean Fever / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000235:genetics; D006801:Humans; D008297:Male; D020125:Mutation, Missense; D000071198:Pyrin; D047888:Receptors, Tumor Necrosis Factor, Type I / Q000235:genetics; D050257:Tubulin Modulators / Q000627:therapeutic use*
chemical_list: D003598:Cytoskeletal Proteins; C000604192:MEFV protein, human; D000071198:Pyrin; D047888:Receptors, Tumor Necrosis Factor, Type I; D050257:Tubulin Modulators; D003078:Colchicine
keywords: familial Mediterranean fever; hereditary autoinflammatory diseases; polyarteritis nodosa; tumor necrosis factor receptor-associated periodic syndrome; vasculitis