副肿瘤性天疱疮伴发Castleman's病和间质性肺炎  

Paraneoplastic pemphigus associated with castleman's disease and interstitial pneumonia

作　　者：汪明华[1] 解芳[1] 樊建峰[1] 李恒进[1] 

机构地区：[1]中国人民解放军总医院,北京100853

出　　处：《山东医药》2008年第15期32-34,共3页Shandong Medical Journal

基　　金：国家自然科学基金资助项目(30470957)

摘　　要：目的总结首例伴发Castleman's病和间质性肺炎的副肿瘤性天疱疮(PNP)的诊治经验。方法结合患者临床表现和组织病理、免疫病理和影像学检查结果诊断为PNP伴Castleman's病和间质性肺炎。用大剂量丙种球蛋白中和抗体,手术切除肿瘤,用大剂量激素冲击疗法治疗间质性肺炎。结果患者临床症状均缓解,随访至今无复发。结论PNP不但与内脏肿瘤相关,也可累及呼吸系统;手术切除、大剂量丙种球蛋白及激素冲击疗法是该病的有效治疗方法。Objective To summarize the clinical experience of the first case of paraneoplastic pemphigus （PNP） associated with Casfleman＇s disease and interstitial pneumonia. Methods According to clinical presentations and laboratory tests of histopathology, immunopathology and imageology, PNP associated with Castleman＇s disease and interstitial pneumonia was diagnosed. The patient was treated in high dose gamma globulin to neutralize antibodies, exairesis of tumor and glucocorticoid stosstherapy for interstitial pneumonia. Results Symptoms of the patient were relieved. No recurrence has been found with the follow-up visit until now. Conclusion PNP is not only associated with visceral malignancy, but also with respiratory system disease. The effective therapies of the disease are exairesis, high dose gamma globtdin and glucoeorticoid stosstherapy.

关 键 词：副肿瘤性天疱疮 Castleman's病 间质性肺炎 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]