Lichen Sclerosus: A Current Landscape of Autoimmune and Genetic Interplay.
Lichen Sclerosus: A Current Landscape of Autoimmune and Genetic Interplay.
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DOI：
10.3390/diagnostics12123070
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发表时间：
2022-12-06
期刊：
影响因子：
3.6
通讯作者：
Hasegawa, Minoru
中科院分区：
文献类型：
--
作者：
Oyama, Noritaka;Hasegawa, Minoru
关键词：
硬化性苔藓(LS)是一种获得性慢性炎症性皮肤病，主要累及肛门生殖器部位，伴有顽固性瘙痒和疼痛。进行性或持续性LS可能会导致排尿和性功能障碍，并增加局部皮肤恶性肿瘤的风险，患病率高达11%。类脂蛋白沉积症是一种常染色体隐性遗传性皮肤病，由细胞外基质蛋白1(ECM1)基因的功能缺失突变引起。研究发现，LS对同一分子存在体液自身免疫反应，为针对ECM1的自身免疫和遗传对应物提供了证据。本文概述了更好地了解LS中ECM1的免疫病理机制的基本重要性和当前的问题。此外，我们强调ECM1在皮肤生物学的动态平衡和结构维持以及可能与ECM1功能受损或获得相关的各种人类疾病中的多效性作用，包括炎症性肠病溃疡性结肠炎、Th2细胞依赖的呼吸道过敏、T细胞和B细胞激活以及脱髓鞘中枢神经系统疾病多发性硬化症，以促进分享作为这种有吸引力的分子的合理治疗靶点的概念。
Lichen sclerosus (LS) is an acquired chronic inflammatory dermatosis predominantly affecting the anogenital area with recalcitrant itching and soreness. Progressive or persistent LS may cause urinary and sexual disturbances and an increased risk of local skin malignancy with a prevalence of up to 11%. Investigations on lipoid proteinosis, an autosomal recessive genodermatosis caused by loss-of-function mutations in the extracellular matrix protein 1 (ECM1) gene, led to the discovery of a humoral autoimmune response to the identical molecule in LS, providing evidence for an autoimmune and genetic counterpart targeting ECM1. This paper provides an overview of the fundamental importance and current issue of better understanding the immunopathology attributed to ECM1 in LS. Furthermore, we highlight the pleiotropic action of ECM1 in homeostatic and structural maintenance of skin biology as well as in a variety of human disorders possibly associated with impaired or gained ECM1 function, including the inflammatory bowel disease ulcerative colitis, Th2 cell-dependent airway allergies, T-cell and B-cell activation, and the demyelinating central nervous system disease multiple sclerosis, to facilitate sharing the concept as a plausible therapeutic target of this attractive molecule.
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影响因子：
--
作者：
Kirby, L;Gran, S;Kreuser-Genis, I;Owen, C;Simpson, R
通讯作者：
Simpson, R
DOI：
10.1007/978-3-030-92616-8_7
发表时间：
2022-01-01
期刊：
IMMUNOGENETICS OF DERMATOLOGIC DISEASES
影响因子：
--
作者：
Beigi, Pooya Khan Mohammad
通讯作者：
Beigi, Pooya Khan Mohammad
影响因子：
30.8
作者：
Fisher, Sheila A.;Tremelling, Mark;Anderson, Carl A.;Gwilliam, Rhian;Bumpstead, Suzannah;Prescott, Natalie J.;Nimmo, Elaine R.;Massey, Dunecan;Berzuini, Carlo;Johnson, Christopher;Barrett, Jeffrey C.;Cummings, Fraser R.;Drummond, Hazel;Lees, Charlie W.;Onnie, Clive M.;Hanson, Catherine E.;Blaszczyk, Katarzyna;Inouye, Mike;Ewels, Philip;Ravindrarajah, Radhi;Keniry, Andrew;Hunt, Sarah;Carter, Martyn;Watkins, Nick;Ouwehand, Willem;Lewis, Cathryn M.;Cardon, Lon;Lobo, Alan;Forbes, Alastair;Sanderson, Jeremy;Jewell, Derek P.;Mansfield, John C.;Deloukas, Panos;Mathew, Christopher G.;Parkes, Miles;Satsangi, Jack
通讯作者：
Satsangi, Jack
影响因子：
10.3
作者：
HARRINGTON, CI;DUNSMORE, IR
通讯作者：
DUNSMORE, IR
影响因子：
4.5
作者：
Abdelbaky, Ather M.;Aluru, Pavan;Greene, Damien R.
通讯作者：
Greene, Damien R.