Autoinflammatory syndromes

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

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.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Arthritis, Juvenile / diagnosis
  • Arthritis, Juvenile / drug therapy
  • Arthritis, Juvenile / immunology*
  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / drug therapy
  • Autoimmune Diseases / immunology*
  • Cytokines / therapeutic use*
  • Diagnosis, Differential
  • Fever / drug therapy
  • Fever / immunology
  • Hereditary Autoinflammatory Diseases / diagnosis
  • Hereditary Autoinflammatory Diseases / drug therapy
  • Hereditary Autoinflammatory Diseases / immunology*
  • Humans
  • Immunity, Innate / immunology
  • Inflammation / drug therapy
  • Inflammation / immunology
  • Still's Disease, Adult-Onset / diagnosis
  • Still's Disease, Adult-Onset / drug therapy
  • Still's Disease, Adult-Onset / immunology*
  • Syndrome

Substances

  • Cytokines