›› 2016, Vol. 36 ›› Issue (7): 645-648.

• 综述 • 上一篇    下一篇

副肿瘤性天疱疮的研究进展

吴悠,范媛   

  1. 江苏省口腔医院
  • 收稿日期:2016-02-03 修回日期:2016-03-09 出版日期:2016-07-28 发布日期:2016-07-19
  • 通讯作者: 范媛 E-mail:fanyuan65@hotmail.com
  • 基金资助:
    国家自然科学基金;江苏省高校优势学科建设工程资助项目

Research progress of paraneoplastic pemphigus

You WU1,   

  • Received:2016-02-03 Revised:2016-03-09 Online:2016-07-28 Published:2016-07-19

摘要: 副肿瘤性天疱疮(paraneoplastic pemphigus,PNP)是一种与肿瘤相伴发的累及黏膜、皮肤等多系统的自身免疫性综合征。临床特征为伴发肿瘤的黏膜糜烂及多形性皮肤损害。由于临床表现复杂,首诊误诊率较高,近年来有关该病的发病机制、临床表现等方面有较深入的研究,该文从发病机理、临床表现、组织病理学及免疫学特点、诊断及治疗等方面对PNP进行总结及阐述,以加深对该疾病的认识,提高诊疗效果。

关键词: 副肿瘤性天疱疮, 黏膜糜烂, 诊断

Abstract: Paraneoplastic pemphigus (PNP) is a rare, lifethreatening, autoimmune, and mucocutaneous blistering disease associated with neoplasia. The clinical features of PNP are mucosal erosion and polymorphic skin lesions in patients with concomitant tumor. Due to the complexity of the clinical manifestations, the misdiagnosis rate of the first diagnosis is high. In recent years, the pathogenesis and clinical manifestations of this disease have been more intensively studied. This article expounded and summarized PNP in terms of its pathogenesis, clinical manifestation, pathological and immunological features, as well as diagnosis and treatment to better understand this disease and improve the effect of diagnosis and treatment.

Key words: paraneoplastic pemphigus, mucosal erosion, diagnosis

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