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Dermatol Clin
2013[Jul]; 31
(3
): 471-80
PMID23827249
show ga
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.