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首页> 外文期刊>Clinics in dermatology >Cutaneous manifestations of systemic autoinfiammatory disorders
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Cutaneous manifestations of systemic autoinfiammatory disorders

机译：全身性自动感染性疾病的皮肤表现

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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 and acquired multifactorial disorders, show a significant morbidity and usually persist for life. Due to the limited awareness of sAlDs, they are often associated with a considerable delay in diagnosis. Within the last decade, the use of cytokine-neutralizing therapies has been shown to improve the clinical symptoms of many patients with different sAIDs. Because skin involvement, such as urticarial, pustular, or ulcerative eruptions, is common in a variety of autoinflammatory disorders, dermatologists should be aware of the most important diseases and their skin phenotypes. This review gives an overview on prototype sAIDs with focus on cutaneous manifestations, clinical clues, and diagnostic approaches. Effective treatment options, such as anti interleukin-l targeted therapies, are discussed. (C) 2015 Elsevier Inc. All rights reserved.
机译：罕见的全身性自身炎性疾病（sAID）是由细胞因子介导的不受控制的炎症驱动的，炎症是由先天免疫途径的激活引起的。 sAID表现为反复发烧，疲劳，肌肉骨骼症状，胃肠道，神经系统和皮肤表现。它们包括遗传性单基因遗传性和获得性多因素疾病，显示出高发病率，并且通常终身存在。由于对sAlDs的认识有限，它们通常与诊断的相当大的延迟有关。在过去的十年中，已证明使用细胞因子中和疗法可以改善许多具有不同sAID的患者的临床症状。由于在各种自发性疾病中常见皮肤受累，例如荨麻疹，脓疱或溃疡性爆发，因此皮肤科医生应了解最重要的疾病及其皮肤表型。这篇综述概述了原型sAID，重点关注皮肤表现，临床线索和诊断方法。讨论了有效的治疗选择，例如抗白介素-1靶向治疗。 （C）2015 Elsevier Inc.保留所有权利。

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