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Background
This disease, also known as HIDS, is a rare disroder associated with periodic fever of 3-7 days duration. There may be associated abdominal pain, arthralgia, and lymphadenopathy. There may be a skin rash characterized by small erythematous macules, papules, urticaria, and nodules occurring on the extremities.
SYNONYMS HIDS Dutch, French, or other European
DISEASE ASSOCIATIONS CHARACTERIZATION Sweet's like or cellulitis-like rash Henoch-Schonlein purpura Erythema elevatum diutinum
PATHOGENESIS CHARACTERIZATION Autosomal recessive MVK gene (12q24) Nat Genet 1999;22:178-181
Codes for mevalonate kinase
LABORATORY/RADIOLOGIC/OTHER TESTS CHARACTERIZATION Elevated serum IgD Elevated in 95% of cases
HISTOLOGICAL TYPES CHARACTERIZATION General Diffuse neutrophilic dermatosis VARIANTS
PROGNOSIS AND TREATMENT CHARACTERIZATION Treatment NSAIDs or corticosteroids Ped Infect Dis J 1996;15:72-77.
Ann Rheum Dis 1993;52:81
Last Updated 1/4/2001
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