Document Detail


Cryopyrin-Associated Periodic Syndrome.
MedLine Citation:
PMID:  22891689     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
  Cryopyrin-associated periodic syndromes (CAPS) are characterized by apparently unprovoked attacks of fever, rashes, and musculoskeletal and sensorineural inflammation accompanied by high acute-phase reactants. Excessive interleukin-1 (IL-1) signaling appears to be a constant feature in the pathomechanism of the disease, driven by a gain-of-function mutation in the NLRP3 gene. Herein, we present the case of a 9-month-old boy with recurrent nonpruritic rashes and episodes of fever. The difficulties of early diagnosis due to initially mild clinical symptoms and the dramatic response to anti-IL-1 therapy after diagnosis emphasize the practical relevance of considering CAPS as a differential diagnosis in these patients.
Authors:
Christian Posch; Wilhelm Kaulfersch; Klemens Rappersberger
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-8-14
Journal Detail:
Title:  Pediatric dermatology     Volume:  -     ISSN:  1525-1470     ISO Abbreviation:  Pediatr Dermatol     Publication Date:  2012 Aug 
Date Detail:
Created Date:  2012-8-15     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8406799     Medline TA:  Pediatr Dermatol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2012 Wiley Periodicals, Inc.
Affiliation:
Department of Dermatology and Venereology, Rudolfstiftung Hospital, Vienna, Austria Department of Pediatrics and Adolescent Medicine, General Hospital of Klagenfurt, Klagenfurt, Austria.
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