1例伴发恶性胸腺瘤的副肿瘤性天疱疮临床及免疫学研究

• 作 者: 张秉新 王京 郑瑞 林涛 李挺 王仁贵 卜定方 朱学骏
• 机 构: 北京大学第一医院皮肤科,北京大学第一医院皮肤科,北京大学第一医院皮肤科,北京大学第一医院胸外科,北京大学第一医院病理科,北京大学第一医院影像科,北京大学第一医院中心实验室,北京大学第一医院皮肤科 北京
• 出 处: 中国皮肤性病学杂志
• 发表日期: 2004年11期
• 关键词: 副肿瘤性天疱疮 恶性胸腺瘤 自身抗原

目的报告我国首例伴发恶性胸腺瘤的副肿瘤性天疱疮(PNP)临床及免疫学特征。方法对患者进行系统体检、医学影像学、组织病理学和免疫学检查,使用重组蛋白免疫印迹法对由胸腺瘤分泌抗体所针对的自身抗原进行了研究。结果患者确诊为PNP,伴发恶性胸腺瘤,患者血清中含有抗重组人包斑蛋白(envoplakin)和周斑蛋白(periplakin)L亚区GST融合蛋白的抗体。结论PNP具有独特的临床表现、组织病理以及免疫学特点,患者血清中的自身抗体针对多种抗原,包斑蛋白和周斑蛋白的L亚区很可能是PNP患者血清抗体结合的主要位点。

Objective To report the clinical and immunological characteristics of the first paraneoplastic pemphigus (PNP) with malignant thymoma in China. Methods The physical examination, medical imaging, histopathology and immunology examination were performed on the patients. The autoantigen against which the antibodies were secreted by thymoma was studied by using recombinant protein immunoblotting. Results The patient was diagnosed as PNP with malignant thymoma, and the serum of the patient contained anti-recombinant envoplakin and periplakin L sub-region GST fusion protein. Conclusions PNP has a unique clinical manifestation, histopathology and immunological features. Serum autoantibodies against many antigens, P53 and P53 are likely to be the main sites of antibody binding in PNP patients.