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一名模拟中毒性表皮坏死松解症的百岁男性患大疱性类天疱疮

Bullous Pemphigoid in a Centenarian Male Simulating Toxic Epidermal Necrolysis.

作者信息

Shakhashiro Muna, Bradley Taylor R, Tobin Stuart

机构信息

Dermatology, University of Kentucky College of Medicine, Lexington, USA.

Dermatology, Lexington Veterans Affairs (VA) Health Care System, Lexington, USA.

出版信息

Cureus. 2023 Sep 11;15(9):e45037. doi: 10.7759/cureus.45037. eCollection 2023 Sep.

DOI:10.7759/cureus.45037
PMID:37829935
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10566394/
Abstract

Bullous pemphigoid (BP) is one of the most common autoimmune blistering diseases and classically presents as large, tense bullae. We report a case of BP with toxic epidermal necrolysis (TEN)-like manifestations in a 103-year-old male, the oldest known patient to present with an acute onset of BP. Our patient presented with extensive erosive lesions comprising 12% of the total body surface area, raising suspicion of TEN and Staphylococcal scalded skin syndrome. Detailed clinical, histological, and immunofluorescence analyses were performed, confirming a diagnosis of BP. Atypical presentations of blistering disorders can be a diagnostic challenge and require the use of histologic and direct immunofluorescence testing to distinguish between clinically similar cutaneous diseases. Proper diagnosis is essential to ensure appropriate management and patient care.

摘要

大疱性类天疱疮(BP)是最常见的自身免疫性水疱病之一,典型表现为大的、紧张性水疱。我们报告一例103岁男性的BP病例,其具有中毒性表皮坏死松解症(TEN)样表现,是已知急性起病的BP患者中年龄最大的。我们的患者出现广泛的糜烂性皮损,占体表面积的12%,这引发了对TEN和葡萄球菌性烫伤样皮肤综合征的怀疑。进行了详细的临床、组织学和免疫荧光分析,确诊为BP。水疱性疾病的非典型表现可能是诊断上的挑战,需要使用组织学和直接免疫荧光检测来区分临床相似的皮肤病。正确诊断对于确保适当的管理和患者护理至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/47b5/10566394/d37ea65c4755/cureus-0015-00000045037-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/47b5/10566394/c8423a730230/cureus-0015-00000045037-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/47b5/10566394/d37ea65c4755/cureus-0015-00000045037-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/47b5/10566394/c8423a730230/cureus-0015-00000045037-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/47b5/10566394/d37ea65c4755/cureus-0015-00000045037-i02.jpg

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本文引用的文献

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Bullous pemphigoid secondary to pembrolizumab mimicking toxic epidermal necrolysis.帕博利珠单抗继发大疱性类天疱疮,酷似中毒性表皮坏死松解症。
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Atypical presentations of bullous pemphigoid: Clinical and immunopathological aspects.大疱性类天疱疮的不典型表现:临床和免疫病理方面。
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Toxic epidermal necrolysis: Part I. Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis.
中毒性表皮坏死松解症:第一部分。引言、历史、分类、临床特征、全身表现、病因和免疫发病机制。
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