Home Diseases and Health Information  

Home Home Translating Report News Physicians Diseases Body Sites Diseases and Health Information Search

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
GEOGRAPHY
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


Commonly Used Terms

Skin


Last Updated 1/4/2001

Send mail to psdermpath@earthlink.net with questions or comments about this web site.
Copyright 2001 The Doctor's Doctor