Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever.

[familial mediterranean fever]

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 M 694 I 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 .

Diseases
Validation

Diseases presenting "fever" symptom

22q11.2 deletion syndrome

acute rheumatic fever

alexander disease

allergic bronchopulmonary aspergillosis

canavan disease

carcinoma of the gallbladder

child syndrome

congenital toxoplasmosis

cushing syndrome

cystinuria

dracunculiasis

erdheim-chester disease

esophageal adenocarcinoma

esophageal carcinoma

familial mediterranean fever

focal myositis

hodgkin lymphoma, classical

lamellar ichthyosis

legionellosis

locked-in syndrome

malignant atrophic papulosis

neonatal adrenoleukodystrophy

neuralgic amyotrophy

oculocutaneous albinism

papillon-lefèvre syndrome

pyomyositis

pyruvate dehydrogenase deficiency

scrub typhus

severe combined immunodeficiency

sneddon syndrome

systemic capillary leak syndrome

triple a syndrome

typhoid

waldenström macroglobulinemia

wolf-hirschhorn syndrome

This symptom has already been validated