中华皮肤科杂志 ›› 2005, Vol. 38 ›› Issue (12): 745-747.

• 论著 • 上一篇    下一篇

伴发副肿瘤性天疱疮的Castleman瘤——附10例报告

朱学骏1, 王京1, 陈喜雪1, 王仁贵2, 张澜波3, 李挺4, 王爱平1, 杨淑霞1, 涂平1, 李若瑜1, 吴艳1, 杨海珍1, 季素珍1   

  1. 1. 北京大学第一医院皮肤性病科 100034;
    2. 北京大学第一医院医学影像科;
    3. 北京大学第一医院普通外科;
    4. 北京大学第一医院病理科
  • 收稿日期:2004-07-26 发布日期:2005-12-15
  • 基金资助:
    国家自然科学基金资助项目(30371292);2003年度高校博士点专项科研基金(20030001021)

Castleman Tumor in Association with Paraneopiastic Pemphigus-A Report of 10 Cases

ZHU Xue-jun1, WANG Jing1, CHEN Xi-xue1, WANG Ren-gui2, ZHANG Lan-bo3, LI Ting4, WANG Ai-ping1, YANG Shu-xia1, TU Ping1, LI Ruo-yu1, WU Yan1, YANG Hai-zhen1, JI Su-zhen1   

  1. Department of Dermatology, Peking University First Hospital, Beijing 100034, China
  • Received:2004-07-26 Published:2005-12-15

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摘要： 目的 了解Castleman瘤伴发副肿瘤性天疱疮的临床特点。方法 分析总结我科诊治的10例Castleman瘤伴发副肿瘤性天疱疮的临床特点和治疗方法。结果 Castleman瘤为我国最常见伴发副肿瘤性天疱疮的肿瘤,其临床表现、皮损和肿瘤的病理学特点、CT及免疫学特点都较独特。肿瘤切除手术中给予静脉滴注丙种球蛋白,对降低术后呼吸道症状的出现,使患者早日康复是有益的。结论 由于肿瘤直接产生抗体致病,因此早期诊断、早期切除肿瘤,是成功治疗本病的关键。

关键词: 副肿瘤性天疱疮, 巨淋巴结增生

Abstract: Objective To obtain a better understaning of the clinical features of Castleman tumor associated paraneoplastic pemphigus. Methods The clinical features and therapy of 10 cases of this disease, diagnosed in the Department of Dermatology of Peking University First Hospital were analyzed. Results Castleman tumor was shown to be the most common neoplasm associated with paraneoplastic pemphigus in China. The clinical presentations, histopathologic characteristics, CT scan findings, and immunologic features were all unique. The early diagnosis and removal of the Castleman tumor are crucial for the treatment of this tumor-associated autoimmune disease. Conclusions Because Castleman tumor is directly related to the induction of autoimmunity, early diagnosis and prompt removal of the tumor are essential to the management of this disease.

Key words: Paraneoplastic Pemphigus, Giant lymph node hyperplasia