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 "skin lesions" symptom

child syndrome

cowden syndrome

cutaneous mastocytosis

cystinuria

dystrophic epidermolysis bullosa

epidermolysis bullosa simplex

erdheim-chester disease

erythropoietic protoporphyria

familial mediterranean fever

focal myositis

gm1 gangliosidosis

heparin-induced thrombocytopenia

hirschsprung disease

junctional epidermolysis bullosa

lamellar ichthyosis

liposarcoma

lymphangioleiomyomatosis

malignant atrophic papulosis

oligodontia

omenn syndrome

papillon-lefèvre syndrome

primary effusion lymphoma

proteus syndrome

severe combined immunodeficiency

sneddon syndrome

waldenström macroglobulinemia

werner syndrome

wiskott-aldrich syndrome

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