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首页> 外文期刊>Clinics in dermatology >Pemphigus: Etiology, pathogenesis, and inducing or triggering factors: Facts and controversies
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Pemphigus: Etiology, pathogenesis, and inducing or triggering factors: Facts and controversies

机译：天疱疮：病因，发病机制以及诱发或触发因素：事实与争议

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Pemphigus includes a group of autoimmune bullous diseases with intraepithelial lesions involving the skin and Malpighian mucous membranes. Pemphigus vulgaris (PV), the most frequent and representative form of the group, is a prototypical organ-specific human autoimmune disorder with a poor prognosis in the absence of medical treatment. The pathomechanism of PV hinges on autoantibodies damaging cell-cell cohesion and leading to cell-cell detachment (acantholysis) of the epidermis and Malpighian mucosae (mainly oral mucosa). A controversy exists about which subset of autoantibodies is primarily pathogenic: the desmoglein-reactive antibodies or those directed against the acetylcholine receptors of the keratinocyte membrane.The onset and course of PV depend on a variable interaction between predisposing and inducing factors. Genetic predisposition has a complex polygenic basis, involving multiple genetic loci; however, the genetic background alone ("the soil"), although essential, is not by itself sufficient to initiate the autoimmune mechanism, as proven by the reports of PV in only one of two monozygotic twins and in only two of three siblings with an identical PV-prone haplotype. The intervention of inducing or triggering environmental factors ("the seed") seems to be crucial to set off the disease. The precipitating factors are many and various, most of them directly originating from the environment (eg, drug intake, viral infections, physical agents, contact allergens, diet), others being endogenous (eg, emotional stress, hormonal disorders) but somehow linked with the subject's lifestyle. As to certain drugs, their potential of provoking acantholysis may be implemented by their interfering with the keratinocyte membrane biochemistry (biochemical acantholysis) and/or with the immune balance (immunologic acantholysis). Viral infections, especially the herpetic ones, may trigger the outbreak of PV or simply complicate its clinical course. The precipitating effect might be due to interferons and other cytokines released by the host as a consequence of the viral attack, which overactivate the immune response. Inductions of PV by physical agents (ultraviolet or ionizing radiation, thermal or electrical burns, surgery and cosmetic procedures), contact allergens (in particular, organophosphate pesticides), dietary factors (eg, garlic, leek, onion, black pepper, red chili pepper, red wine, tea), and emotional stress are rare, but well-documented events.The possible intervention of the environment in the outbreak of PV has been overlooked in the past, but nowadays clinicians perceive it more frequently. The assumption that genetic factors alone are not sufficient to cause the outbreak of the disease, inevitably instills the idea that PV may not occur spontaneously, but always results from an interaction between an individual predisposing genetic background and environmental precipitating factors, often concealed or apparently harmless.
机译：天疱疮包括一组自身免疫性大疱性疾病，其上皮内病变累及皮肤和马尔皮基粘膜。寻常型天疱疮（PV）是该组中最常见和最具代表性的形式，是一种原型器官特异性人类自身免疫性疾病，在没有药物治疗的情况下预后较差。 PV的致病机制取决于自身抗体，这些抗体会破坏细胞间的凝聚力，并导致表皮和Malpighian黏膜（主要是口腔黏膜）的细胞脱离（脱落）。关于哪种自身抗体主要致病性存在争议：桥粒芯蛋白反应性抗体或针对角质形成细胞膜乙酰胆碱受体的抗体.PV的发作和进程取决于诱发因素和诱导因素之间的可变相互作用。遗传易感性具有复杂的多基因基础，涉及多个遗传位点。然而，仅遗传背景（“土壤”）虽然必不可少，但其本身不足以启动自身免疫机制，如PV报道的两个单卵双胞胎双胞胎中的一个和三个同卵双胞胎中只有两个的PV所证明的那样。相同的PV倾向单倍型。诱导或触发环境因素（“种子”）的干预似乎对于引发该疾病至关重要。诱发因素多种多样，其中大多数直接来自环境（例如，药物摄入，病毒感染，物理因素，接触性过敏原，饮食），其他是内源性的（例如，情绪压力，荷尔蒙紊乱），但与某种因素相关受试者的生活方式。对于某些药物，可能通过干扰角质形成细胞膜生物化学（生化棘皮分解）和/或免疫平衡（免疫棘皮分解）来发挥其激发棘皮溶解的潜力。病毒感染，尤其是疱疹感染，可能会触发PV的爆发或使其临床进程复杂化。沉淀作用可能是由于病毒攻击导致宿主释放的干扰素和其他细胞因子所致，从而过度激活了免疫反应。物理因素（紫外线或电离辐射，热或电灼伤，手术和美容程序），接触性过敏原（特别是有机磷酸酯农药），饮食因素（例如大蒜，韭菜，洋葱，黑胡椒，红辣椒）诱发的PV ，红酒，茶）和情绪紧张的情况很少见，但有据可查。在过去，人们忽视了环境对PV爆发的可能干预，但如今，临床医生更经常地意识到这一点。仅靠遗传因素不足以引起疾病爆发的假设不可避免地灌输了PV可能不会自发发生，而是总是由个体易感遗传背景和环境诱因（通常被掩盖或看似无害）之间相互作用而产生的观点。 。

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