自身炎性疾病(AID)起因于改变免疫细胞和组织的相互作用的紊乱。它们在不存在异常自身抗体和/或自身反应性T细胞的情况下产生显著的(自身)炎症。主要由炎性小体途径的变化引起的艾滋病，例如NLRP3-或吡喃蛋白相关的炎性体，在过去的几年里获得了大量的关注。然而,主要由先天免疫系统的防御系统的其他变化引起的AID研究较少。这些非炎性体介导的艾滋病与，例如,TNF或IFN信号通路的紊乱或影响IL-1RA的基因的异常。这些病症的临床体征和症状的范围是巨大的。因此，识别早期皮肤体征是皮肤科医生和其他医生鉴别诊断的重要一步。本文就其发病机制进行综述,临床表现,和可用的治疗方案突出了非炎性体介导的AIDs的皮肤病学方面。
Autoinflammatory diseases (AIDs) arise from disturbances that alter interactions of immune cells and tissues. They give rise to prominent (auto)inflammation in the absence of aberrant autoantibodies and/or autoreactive T cells. AIDs that are predominantly caused by changes in the inflammasome pathways, such as the NLRP3- or pyrin-associated inflammasome, have gained substantial attention over the last years. However, AIDs resulting primarily from other changes in the defense system of the innate immune system are less well-studied. These noninflammasome-mediated AIDs relate to, for example, disturbance in the TNF or IFN signaling pathways or aberrations in genes affecting the IL-1RA. The spectrum of clinical signs and symptoms of these conditions is vast. Thus, recognizing early cutaneous signs constitutes an important step in differential diagnoses for dermatologists and other physicians. This review provides an overview of the pathogenesis, clinical presentation, and available treatment options highlighting dermatologic aspects of noninflammasome-mediated AIDs.
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