PDF(Sci-hub)
Abstract：
Pemphigus vulgaris is an autoimmune blistering disease with an increased potential for mortality. The epithelium is key in understanding the pathobiology as it is specialized to perform functions like mechanical protection, immunological defense, and proprioception. In order to perform these array of functions, epithelial integrity is important. This integrity is maintained by a host of molecules which orchestrate the ability of the keratinocytes to function as a single unit. Desmoglein 3 antibodies formed in genetically susceptible individuals are known to cause the disruption of the intact oral mucosa leading to the formation of blisters in pemphigus vulgaris patients. However, there are underlying complex triggering pathways leading to the clinical disease. The aim of the review is to congregate and critically appraise the various triggering pathways which contribute toward the pathobiology of pemphigus vulgaris. Articles relevant to the pathobiology of pemphigus vulgaris were identified from various search databases till the year 2020. The pathogenesis of pemphigus vulgaris is complex, and it involves an in-depth understanding of the various predisposing factors, provoking factors, and progression mechanisms. Congregation of the various triggering pathways will open our minds to understand pemphigus vulgaris better and in turn develop a reliable treatment in the near future.
摘要：
寻常型天疱疮是一种自身免疫性起泡疾病，死亡率增加。上皮是理解病理生物学的关键，因为它是专门执行功能，如机械保护，免疫防御,和本体感受。为了执行这些功能，上皮完整性很重要。这种完整性由协调角质形成细胞作为单个单元发挥作用的能力的大量分子维持。已知在遗传易感个体中形成的Desmoglein3抗体会导致完整的口腔粘膜破坏，从而导致寻常型天疱疮患者水疱的形成。然而,存在导致临床疾病的潜在复杂触发途径。这篇综述的目的是聚集并批判性地评估有助于寻常型天疱疮病理生物学的各种触发途径。直到2020年，从各种搜索数据库中确定了与寻常型天疱疮病理生物学相关的文章。寻常型天疱疮的发病机制复杂,它涉及对各种诱发因素的深入理解，挑衅因素,和进展机制。各种触发途径的聚集将打开我们的思想，更好地理解寻常型天疱疮，并在不久的将来开发出可靠的治疗方法。
