Autoinflammatory syndromes

Research output: Contribution to journalReview articlepeer-review

19 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)471-480
Number of pages10
JournalDermatologic Clinics
Volume31
Issue number3
DOIs
StatePublished - Jul 2013
Externally publishedYes

Keywords

  • Autoinflammatory
  • CAPS
  • Periodic fever
  • Still disease

ASJC Scopus subject areas

  • Dermatology

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