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一例大疱性类天疱疮:一种常见且可能致命的疾病。

A Case of Bullous Pemphigoid: A Prevalent and Potentially Fatal Condition.

作者信息

Parellada Jorge, Olivera Arencibia Yanetsy, Watson Harold, Parellada Nicole, Saikaly Lara E, Saikaly Sami K

机构信息

Internal Medicine, Orlando Regional Medical Center.

School of Medicine, Geisinger Commonwealth School of Medicine.

出版信息

Cureus. 2018 Apr 25;10(4):e2533. doi: 10.7759/cureus.2533.

Abstract

Bullous pemphigoid is the most common of the blistering disorders. It is most commonly found in the elderly and is diagnosed based on clinical, histologic, and immunologic criteria. It presents clinically with diffuse eczematous, pruritic, urticaria-like lesions, with the later appearance of tense bullae or blistering lesions typically filled with clear fluid. Histologically, a sub-epidermal blister is seen and immunofluorescence shows immunoglobulin G antibodies directed against the structural components of the keratinocytic hemidesmosomal proteins BP180 and BP230. Multiple treatment modalities are present for this condition, including anti-inflammatory medications, medications that reduce antibody formation, and treatments to increase the elimination of antibodies. The aim of this case report is to present a classic case of this condition, to highlight an awareness of differing treatment options, and to advocate referral to a dermatologist given its potential severity.

摘要

大疱性类天疱疮是最常见的水疱性疾病。它最常见于老年人,根据临床、组织学和免疫学标准进行诊断。临床上表现为弥漫性湿疹样、瘙痒性、荨麻疹样皮损,随后出现紧张性大疱或水疱性皮损,通常充满清亮液体。组织学上可见表皮下水疱,免疫荧光显示针对角质形成细胞半桥粒蛋白BP180和BP230结构成分的免疫球蛋白G抗体。针对这种疾病有多种治疗方式,包括抗炎药物、减少抗体形成的药物以及增加抗体清除的治疗方法。本病例报告的目的是呈现该疾病的一个典型病例,强调对不同治疗选择的认识,并鉴于其潜在的严重性提倡转诊至皮肤科医生处。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f2fb/6017127/a977e7c1ce11/cureus-0010-00000002533-i01.jpg

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