Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever |
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| Authors: | Shigetsuna Komatsu Masaru Honma Satomi Igawa Hitomi Tsuji Akemi Ishida‐Yamamoto Kiyoshi Migita Hiroaki Ida Hajime Iizuka |
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| Institution: | 1. Department of Dermatology, Asahikawa Medical University, , Asahikawa, Japan;2. Clinical Research Center, Nagasaki Medical Center, , Omura, Nagasaki, Japan;3. Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine, , Kurume, Japan |
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| 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. |
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| Keywords: | familial Mediterranean fever hereditary autoinflammatory diseases polyarteritis nodosa tumor necrosis factor receptor‐associated periodic syndrome vasculitis |
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