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"Autoinflammatory psoriasis"-genetics and biology of pustular psoriasis.
Cellular & Molecular Immunology ( IF 21.8 ) Pub Date : 2020-08-19 , DOI: 10.1038/s41423-020-0519-3
Ranjitha Uppala 1, 2 , Lam C Tsoi 2 , Paul W Harms 2, 3 , Bo Wang 2 , Allison C Billi 2 , Emanual Maverakis 4 , J Michelle Kahlenberg 5, 6 , Nicole L Ward 7 , Johann E Gudjonsson 2, 6
Affiliation  

Psoriasis is a chronic inflammatory skin condition that has a fairly wide range of clinical presentations. Plaque psoriasis, which is the most common manifestation of psoriasis, is located on one end of the spectrum, dominated by adaptive immune responses, whereas the rarer pustular psoriasis lies on the opposite end, dominated by innate and autoinflammatory immune responses. In recent years, genetic studies have identified six genetic variants that predispose to pustular psoriasis, and these have highlighted the role of IL-36 cytokines as central to pustular psoriasis pathogenesis. In this review, we discuss the presentation and clinical subtypes of pustular psoriasis, contribution of genetic predisposing variants, critical role of the IL-36 family of cytokines in disease pathophysiology, and treatment perspectives for pustular psoriasis. We further outline the application of appropriate mouse models for the study of pustular psoriasis and address the outstanding questions and issues related to our understanding of the mechanisms involved in pustular psoriasis.



中文翻译:

“自身炎症性银屑病”-脓疱性银屑病的遗传学和生物学。

牛皮癣是一种慢性炎症性皮肤病,具有相当广泛的临床表现。斑块型银屑病是银屑病最常见的表现,位于光谱的一端,以适应性免疫反应为主,而罕见的脓疱型银屑病则位于另一端,以先天性和自身炎症免疫反应为主。近年来,遗传学研究已经确定了六种易患脓疱型银屑病的遗传变异,这些变异突出了 IL-36 细胞因子在脓疱型银屑病发病机制中的作用。在这篇综述中,我们讨论了脓疱型银屑病的表现和临床亚型、遗传易感变异的贡献、细胞因子 IL-36 家族在疾病病理生理学中的关键作用以及脓疱型银屑病的治疗前景。

更新日期:2020-08-19
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