副肿瘤性天疱疮伴发Castleman病三例分析  

Paraneoplastic pemphigus in association with Castleman tumor:a report of 3 cases

作　　者：赵慧霞[1,2] 王彩虹 贾振宇[2] 罗静 李小峰 张艳玲[3] 

机构地区：[1]山西医科大第二医院风湿免疫科,太原030001 [2]长治医学院附属和济医院皮肤科 [3]长治医学院附属和济医院呼吸科

出　　处：《中国药物与临床》2011年第10期1124-1126,1233,共3页Chinese Remedies & Clinics

基　　金：山西省科技攻关项目(20110313013-1);山西省教育厅科技项目(20111107)

摘　　要：目的探讨副肿瘤性天疱疮伴发Castleman病的临床特点。方法回顾性分析3例副肿瘤性天疱疮伴发Castleman病的临床特征和治疗方法。结果副肿瘤性天疱疮多由Castleman病诱发,其临床表现、皮损和肿瘤的病理学特点、CT及免疫学特点较特殊,易于明确诊断,切除肿瘤对治疗副肿瘤性天疱疮伴发Castleman病、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Castleman病直接产生抗体致副肿瘤性天疱疮,因此早期诊断、早期切除肿瘤是治疗本病的关键,如果合并闭塞性细支气管炎,预后较差,病死率高。Objective To investigate the clinical features of Castleman tumor associated with paraneoplastic pemphigus.Methods The clinical features and therapy for 3 cases of Castleman tumor associated with paraneoplastic pemphigus were analyzed retrospectively.Results Paraneoplastic pemphigus in these cases was induced by Castleman tumor,with specific clinical presentations,histopathologic characteristics,CT findings,and immunologic features which may facilitate a confirmed diagnosis.Tumor resection appeared to show benefits for patients with this condition in reducing respiratory symptoms and expediting an early recovery.Conclusion Since Castleman tumor may result in production of antibodies which frequently give rise to paraneoplastic pemphigus,early diagnosis and prompt removal of the tumor are essential to the management of this disease.Development of obliterative bronchiolitis in patients would lead to poor prognosis and high fatality.The 3 cases reported in this study were not complicated with such a condition,therefore indicating a better outcome.

关 键 词：巨淋巴结增生 天疱疮 

分 类 号：R758.6[医药卫生—皮肤病学与性病学]