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烧伤重症监护病房中的副肿瘤性天疱疮:病例报告及文献复习

Paraneoplastic pemphigus in a burn intensive care unit: case report and review of the literature.

作者信息

Hayanga Awori J, Lee Timothy M, Pannucci Christopher J, Knipp Brian S, Olsen Stephen H, Wang Stewart C, Napolitano Lena M

机构信息

Department of Surgery, University of Michigan, Ann Arbor, Michigan 48109, USA.

出版信息

J Burn Care Res. 2010 Sep-Oct;31(5):826-9. doi: 10.1097/BCR.0b013e3181eed4b4.

Abstract

Paraneoplastic pemphigus is a rare cause of acute diffuse blistering in the adult patient. It commonly presents with subepidermal blistering, epidermal necrosis, and symptoms of mucosal irritation, such as conjunctivitis and vaginal ulceration. Because of its rarity, it is frequently misdiagnosed as Stevens-Johnson syndrome or toxic epidermal necrolysis. In this study, the authors will describe clinical and histologic manifestations of paraneoplastic pemphigus. This case report describes a 45-year-old woman with paraneoplastic pemphigus who was admitted and treated in a burn intensive care unit. Although initially diagnosed with Stevens-Johnson syndrome, the patient had progression of desquamation when potentially offending medications were discontinued. Diffuse adenopathy was noted on examination, and biopsy confirmed a low-grade lymphoma. Paraneoplastic pemphigus is a rare but important cause of acute diffuse blistering in adults. This disorder should be considered in the differential diagnosis of patients with diffuse blistering.

摘要

副肿瘤性天疱疮是成年患者急性弥漫性水疱形成的罕见原因。它通常表现为表皮下水疱形成、表皮坏死以及黏膜刺激症状,如结膜炎和阴道溃疡。由于其罕见性,它常被误诊为史蒂文斯 - 约翰逊综合征或中毒性表皮坏死松解症。在本研究中,作者将描述副肿瘤性天疱疮的临床和组织学表现。本病例报告描述了一名45岁患有副肿瘤性天疱疮的女性,她被收治在烧伤重症监护病房。尽管最初被诊断为史蒂文斯 - 约翰逊综合征,但在停用可能致病的药物后,患者出现了脱皮进展。检查发现有弥漫性淋巴结病,活检证实为低度淋巴瘤。副肿瘤性天疱疮是成人急性弥漫性水疱形成的一种罕见但重要的原因。在弥漫性水疱形成患者的鉴别诊断中应考虑这种疾病。

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