Cutaneous manifestations of systemic autoinflammatory disorders.

摘要: Rare systemic autoinflammatory diseases (sAIDs) are driven by cytokine-mediated uncontrolled inflammation that results from activation of innate immune pathways. sAIDs present with recurrent fever episodes, fatigue, musculoskeletal symptoms, gastrointestinal, neurologic, and skin manifestations. They include hereditary monogenic acquired multifactorial disorders, show a significant morbidity usually persist for life. Due to the limited awareness sAIDs, they often associated considerable delay in diagnosis. Within last decade, use cytokine-neutralizing therapies has been shown improve clinical symptoms many patients different sAIDs. Because involvement, such as urticarial, pustular, or ulcerative eruptions, is common variety dermatologists should be aware most important their phenotypes. This review gives an overview on prototype focus cutaneous manifestations, clues, diagnostic approaches. Effective treatment options, anti-interleukin-1-targeted therapies, discussed.