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Review
. 2013 Jul;31(3):471-80.
doi: 10.1016/j.det.2013.05.001.

Autoinflammatory syndromes

John J Cush  1
Affiliations
Review

Autoinflammatory syndromes

John J Cush. Dermatol Clin. 2013 Jul.

Abstract

Autoinflammatory syndromes comprise a diagnostically challenging group of systemic inflammatory disorders uniquely related by (1) dysregulation of innate immunity, (2) inflammasome activation, (3) dramatic clinical features (high fevers, neutrophilic rashes, and bone or synovial involvement), (4) impressive acute phase responses, and (5) effective treatment with cytokine inhibitors. This review details some of the more common autoinflammatory disorders, their distinguishing features and dermatologic manifestations, and how an accurate diagnosis can be established in patients presenting with periodic or intermittent febrile disorders.

Keywords: Autoinflammatory; CAPS; Periodic fever; Still disease.

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Figures

Fig. 1
Fig. 1
The approach to undiagnosed periodic fever.
See this image and copyright information in PMC

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