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副肿瘤天疱疮临床和发病机理研究的进展
发表时间：2008-02-15 浏览量：2314 下载量：639
| 全部作者： | 王京，朱学骏 |
| 作者单位： | 北京大学第一医院；北京大学第一医院 |
| 摘 要： | 副肿瘤性天疱疮（PNP）是由Anhalt等人于1990年命名的一种自身免疫性皮肤黏膜疾病，以伴发肿瘤及血清中存在能识别表皮蛋白成分独特的IgG自身抗体为特点。本文就笔者所在单位近年诊治的12例病人的临床特点进行总结，综述了本病发病机理研究的几种主要学说的特点和主要缺陷，总结了探索自身抗体产生的机制进行的深入、细致的研究的主要发现。对PNP伴发的Castleman瘤B细胞克隆的免疫遗传学研究表明肿瘤B细胞具有产生抗体的能力和结构基础，进一步总结出Castleman瘤能直接产生自身抗体是致病的关键。这一发现的意义在于对PNP的治疗有重大指导价值，这种理论可以引申用于体液免疫介导的自身免疫疾病的研究和治疗。 |
| 关 键 词： | 皮肤病学；发病机理；副肿瘤性天疱疮；肿瘤；自身抗体 |
| Title： | The clinical characteristics and new development on pathogenesis of autoimmune paraneoplastic pemphigus |
| Author： | WANG Jing, ZHU Xuejun |
| Organization： | Department of dermatology, Peking university first hospital |
| Abstract： | Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease firstly reported by Anhalt et al in 1990 . The disease is characterized by distinctive clinical symptoms and signs such as severe, painful mucosal erosions, polymorphous skin lesions, histopathology hallmarks and immunological findings. The clinical findings of 12 PNP cases diagnosed in our department in recent 5 years were analyzed. The hypothesis for the pathogenesis of this disease was reviewed. The relationship between the existence of Castleman’s disease and the autoimmune reaction to the skin is the main challenge of the study. The study to the B cell clones in Castleman’s tumors found that the tumor B cells had the ability to produce antibody. The tumor could secreting autoantibody is the key point of the pathogenesis for the autoimmune reaction of the disease. The new findings were used in the management of the PNP patients and method can be used in the pathogenesis study for other autoimmune diseases. |
| Key words： | dermatology；pathogenesis; paraneoplastic pemphigus; tumor. |
| 发表期数： | 2008年6月第3期 |
| 引用格式： | 王京，朱学骏. 副肿瘤天疱疮临床和发病机理研究的进展[J]. 中国科技论文在线精品论文，2008，1（3）：321-327. |
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