Schnitzler’s syndrome: lessons from 281 cases

Clinical and Translational Allergy

Heleen D de Koning¹ 

[1] Department of Dermatology, Radboud University Medical Center, Nijmegen, The Netherlands

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

Others  :  1092740
DOI  :  10.1186/2045-7022-4-41

 received in 2014-09-19, accepted in 2014-10-19,  发布年份 2014

【 摘 要 】

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.

【 授权许可】

   
2014 de Koning; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

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