中华皮肤科杂志 ›› 2000, Vol. 33 ›› Issue (6): 392-394.

• 论著 • 上一篇    下一篇

副肿瘤性天疱疮二例

王爱平, 朱学骏, 涂平, 马圣清, 李丽   

  1. 北京大学第一医院皮肤科 100034
  • 收稿日期:1999-12-27 出版日期:2000-12-15 发布日期:2000-12-15

Paraneoplastic Pemphigus: A Report of Two Cases

WANG Aiping, ZHU Xuejun, TU Ping   

  1. Department of Dermatology, The First Hospital, Peking University, Beijing 100034
  • Received:1999-12-27 Online:2000-12-15 Published:2000-12-15

摘要: 副肿瘤性天疱疮是一种自身免疫性大疱性疾病,临床表现为严重疼痛性粘膜糜烂和多形性皮损,组织病理学显示棘层松解、苔藓样皮炎或角质形成细胞坏死,存在对各种上皮蛋白的自身抗体,同时伴随潜在肿瘤。本文首次报道2例临床、组织学和免疫病理学均符合副肿瘤性天疱疮病例,伴随Castleman肿瘤。

关键词: 副肿瘤性天疱疮

Abstract: Paraneoplastic pemphigus(PNP) is a clinically, histologically, and immunopathologically distinct autoimmune bullous disease characterized by severe painful mucosal erosions and polymorphous skin lesions. Histopathology shows acantholysis with lichenoid dermatitis or keratinocyte necrosis, and there are autoantibodies to various epidermal proteins with underlying neoplasia. We report two cases of paraneoplastic pemphigus in China firstly. The clinical, histologic, and immunopathologic findings of both cases strongly suggest the diagnosis of paraneoplastic pemphigus associated with Castleman's tumor.

Key words: Paraneoplastic pemphigus