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Published in: Clinical and Translational Allergy 1/2014

Open Access 01-12-2014 | Review

Schnitzler’s syndrome: lessons from 281 cases

Author: Heleen D de Koning

Published in: Clinical and Translational Allergy | Issue 1/2014

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Abstract

Schnitzler’s syndrome is an autoinflammatory disorder characterized by the association of a monoclonal IgM (or IgG) gammopathy, a chronic urticarial rash, and signs and symptoms of systemic inflammation, including fever, arthralgias and bone pain. It was first described in 1972. This review summarizes the clinical features, efficacy of therapies, and follow-up data of the 281 cases that have been reported to date. Also, the results of skin histology, bone imaging, laboratory investigations, and studies of the pathogenesis will be discussed, including the pivotal role of interleukin-1 beta in this disorder.
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Metadata
Title
Schnitzler’s syndrome: lessons from 281 cases
Author
Heleen D de Koning
Publication date
01-12-2014
Publisher
BioMed Central
Published in
Clinical and Translational Allergy / Issue 1/2014
Electronic ISSN: 2045-7022
DOI
https://doi.org/10.1186/2045-7022-4-41

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