Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever. Issue 9 (11th August 2014)

• Record Type: Journal Article
• Authors: Komatsu, Shigetsuna; Honma, Masaru; Igawa, Satomi; Tsuji, Hitomi; Ishida‐Yamamoto, Akemi; Migita, Kiyoshi; Ida, Hiroaki; Iizuka, Hajime
• Is Part Of: Journal of dermatology. Volume 41:Issue 9(2014)
• Page Start: 827
• Page End: 829
• Publication Date: 2014-08-11
• Subjects: Dermatology -- Periodicals; Dermatology -- Japan -- Periodicals; Skin -- Diseases -- Periodicals; 616.5005
• DOI: 10.1111/1346-8138.12588
• Languages: English
• ISSNs: 0385-2407

Abstract

<abstract abstract-type="main" id="jde12588-abs-0001"> <title>Abstract</title> <p>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 <italic>MEFV</italic> 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.</p> </abstract>