13例副肿瘤性天疱疮的临床特征分析

摘要

目的：：明确副肿瘤性天疱疮的临床特点。方法：回顾性分析2004年1月至2013年4月间北京协和医院收治的13例副肿瘤性天疱疮患者的临床资料。结果：13例患者中12例合并肿瘤,其中Castleman病7例,滤泡树突状细胞肉瘤2例,Castleman病合并滤泡树突状细胞肉瘤及指突状树突细胞肉瘤各1例,非霍奇金淋巴瘤( B细胞型)1例。患者均出现外生殖器黏膜溃疡,11例以黏膜溃疡为首发症状,皮损呈多形性。皮损组织病理示：表皮内水疱,基底层液化变性,角质形成细胞坏死。直接免疫荧光( DIF)及间接免疫荧光( IIF)表现为IgG、C3沿棘细胞间和基底膜带沉积。结论：副肿瘤性天疱疮多以黏膜损害为首发表现,皮损表现呈多形性,病理及实验室检查具有特征性,最常伴发的肿瘤是Castleman病,对于经久不愈的黏膜糜烂患者应注意筛查本病以早期诊断及治疗。%Objective:To determine the clinical features of paraneoplastic pemphigus (PNP). Methods:The data of 13 PNP patients in Peking Union Medical College Hospital from January 2004 to April 2013 were analyzed retrospectively. Results:Of the 13 patients, 12 cases were accompanied with cancer, including Cas-tleman disease in 7 cases, follicular dendritic cell sarcoma in 2, Castleman disease and follicular dendritic cell sarcoma in 1 and interdigitating dentrtic cell sarcoma in 1. All the patients had erosion in genital mucosa, which was seen as the first symptom in 11 cases. Biopsy showed blister in epidermis, liquefactive degeneration of basal cells and keratinocyte necrosis. DIF and IIF demonstrated deposition of IgG and C3 throughout the basement membrane zone. Conclusion:The first symptom of PNP is mucosa erosion and the skin lesions are polymorphous. The most common cancer in PNP is Castleman disease. The patients with mucosa erosion should be considered of PNP.