Premium
Cutaneous necrotizing vasculitis as a manifestation of familial M editerranean fever
Author(s) -
Komatsu Shigetsuna,
Honma Masaru,
Igawa Satomi,
Tsuji Hitomi,
IshidaYamamoto Akemi,
Migita Kiyoshi,
Ida Hiroaki,
Iizuka Hajime
Publication year - 2014
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/1346-8138.12588
Subject(s) - medicine , familial mediterranean fever , dermatology , mefv , myalgia , arteritis , vasculitis , colchicine , purpura (gastropod) , cellulitis , gene mutation , pathology , immunology , disease , mutation , gene , ecology , biochemistry , chemistry , biology
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 .