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Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever
Shigetsuna Komatsu1, Masaru Honma, Satomi Igawa
1Department of Dermatology, Asahikawa Medical University, Asahikawa, Japan.
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.
Insights
Familial Mediterranean Fever (FMF), a rare autoinflammatory disease, can manifest with rare cutaneous vasculitis. This case highlights necrotizing arteritis during FMF febrile attacks, successfully treated with colchicine.
Area of Science:
- Rheumatology
- Genetics
- Dermatology
Background:
- Familial Mediterranean Fever (FMF) is an inherited autoinflammatory disorder.
- FMF presents with recurrent fevers, serositis, arthritis, and myalgia.
- Cutaneous manifestations are common, but cutaneous vasculitis is exceptionally rare.
Observation:
- A Japanese patient with sporadic FMF experienced cutaneous arteritis during febrile attacks.
- The patient had a M694I mutation in the MEFV gene.
- Skin lesions included subcutaneous nodules consistent with arteritis.
Findings:
- The case demonstrated a rare association between FMF and cutaneous necrotizing vasculitis.
- Genetic analysis confirmed a heterozygous M694I mutation in the MEFV gene.
- Oral colchicine effectively managed both FMF symptoms and cutaneous arteritis.
Implications:
- Cutaneous necrotizing vasculitis should be considered a potential skin manifestation of FMF.
- This finding expands the spectrum of FMF-associated dermatological conditions.
- Early recognition and treatment of FMF-related vasculitis are crucial for patient outcomes.
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