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Review
. 2014 Dec 5:4:41.
doi: 10.1186/2045-7022-4-41. eCollection 2014.

Schnitzler's syndrome: lessons from 281 cases

Heleen D de Koning  1
Affiliations
Review

Schnitzler's syndrome: lessons from 281 cases

Heleen D de Koning. Clin Transl Allergy. .

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.

Keywords: Autoinflammation; Chronic urticaria; Interleukin-1 beta; Monoclonal gammopathy; Paraprotein; Schnitzler’s syndrome.

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Figure 1
Number of reported cases per country.
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