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大疱性类天疱疮及其他类天疱疮性皮肤病

Bullous Pemphigoid and Other Pemphigoid Dermatoses.

作者信息

Pratasava Valeryia, Sahni Vikram N, Suresh Aishwarya, Huang Simo, Are Abhirup, Hsu Sylvia, Motaparthi Kiran

机构信息

College of Medicine, Drexel University, Philadelphia, PA 19129, USA.

Department of Dermatology, Lewis Katz School of Medicine, Temple University, Philadelphia, PA 19140, USA.

出版信息

Medicina (Kaunas). 2021 Oct 4;57(10):1061. doi: 10.3390/medicina57101061.

Abstract

The pemphigoid family of dermatoses is characterized by autoimmune subepidermal blistering. The classic paradigm for pemphigoid, and the most common member, is bullous pemphigoid. Its variable clinical presentation, with or without frank bullae, is linked by significant pruritus afflicting the elderly. Mucous membrane pemphigoid is an umbrella term for a group of subepidermal blistering dermatoses that favor the mucosal membranes and can scar. Epidermolysis bullosa acquisita is a chronic blistering disorder characterized by skin fragility, sensitivity to trauma, and its treatment-refractory nature. Clinicians that encounter these pemphigoid disorders may benefit from an overview of their clinical presentation, diagnostic work-up, and therapeutic management, with an emphasis on the most frequently encountered pemphigoid disease, bullous pemphigoid.

摘要

类天疱疮家族性皮肤病的特征是自身免疫性表皮下大疱形成。类天疱疮的经典模式以及最常见的类型是大疱性类天疱疮。其临床表现多样,有或无明显水疱,其共同特点是老年患者会出现严重瘙痒。黏膜类天疱疮是一组表皮下大疱性皮肤病的统称,这些疾病好发于黏膜且会形成瘢痕。获得性大疱性表皮松解症是一种慢性大疱性疾病,其特征为皮肤脆弱、对创伤敏感且治疗难治。遇到这些类天疱疮疾病的临床医生可能会从对其临床表现、诊断检查和治疗管理的概述中受益,重点是最常遇到的类天疱疮疾病——大疱性类天疱疮。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/17e0/8539012/dc18a368ea3c/medicina-57-01061-g001.jpg

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