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Schnitzler Syndrome

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Abstract:

A 48-year-old man with typical features of Schnitzler Syndrome is presented, followed by a discussion of the pathogenesis and clinical aspects of this rare disease. Some of the challenges we and others have faced in diagnosing and treating this disease are also discussed. Clinical pearls and pitfalls are emphasized to aid clinicians from varying specialties in recognizing this syndrome and providing appropriate therapy. Schnitzler Syndrome is associated with high morbidity and increased risk of lymphoproliferative disorders. Accurate diagnosis is vital in restoring quality of life and ensuring appropriate long-term monitoring.

Keywords: Anakinra; Schnitzler syndrome; canakinumab; interleukin-1; monoclonal gammopathy; neutrophilic urticarial dermatosis; rituximab; urticaria

Document Type: Research Article

DOI: https://doi.org/10.2500/aap.2014.35.3703

Affiliations: 1School of Medicine, University of Kansas Medical Center, Kansas City, Kansas

Publication date: 2014-01-01

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  • Allergy and Asthma Proceedings is a peer reviewed publication dedicated to distributing timely scientific research regarding advancements in the knowledge and practice of allergy, asthma and immunology. Its primary readership consists of allergists and pulmonologists.

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    The journal is indexed in Thomson Reuters Web of Science and Science Citation Index Expanded, plus the National Library of Medicine's PubMed service.
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