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副肿瘤性天疱疮

Author
Grant J Anhalt, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD
Translators
崔盘根, 主任医师

引言

副肿瘤性天疱疮(paraneoplastic pemphigus, PNP)是一种通常致命性的副肿瘤性皮肤黏膜水疱性疾病，最常由淋巴组织增生性疾病诱发[1]。PNP也称为副肿瘤性自身免疫性多器官综合征(paraneoplastic autoimmune multiorgan syndrome, PAMS)。术语PAMS反映了在PNP情况下可出现非大疱性皮疹及肺部受累的情况[2]。

本文将讨论PNP的发病机制、临床特征及治疗。寻常型天疱疮和落叶型天疱疮见其他专题。 (参见“天疱疮的发病机制、临床表现与诊断”)

流行病学

尽管普遍认为PNP是一种罕见病，但尚不知其具体发病率和患病率。美国食品药品监督管理局(US Food and Drug Administration, FDA)一项纳入了100,000份来自非霍奇金淋巴瘤和慢性淋巴细胞白血病患者的不良事件报告的的回顾性研究，也支持PNP是罕见病[3]。该研究仅确定有12例患者可能存在PNP。

人口统计学表明PNP最常累及的成人年龄段为45-70岁[4]。然而，PNP也可能发生于儿童[5,6]。男女均可受累，尚不确定不同种族、民族或地理环境下发生PNP的风险差异。

病因学

PNP的发生与多种肿瘤性疾病相关。在一项纳入了163例1990-2003年报道的PNP病例的回顾性研究中，与PNP相关的疾病发生率如下[7]：

                               

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Literature review current through: 2017-06 . | This topic last updated: 2017-01-26.
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