副肿瘤性天疱疮11例分析  

Analysis of 11 cases of paraneoplastic pemphigus

作　　者：张燕[1] 潘萌[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院皮肤科,200025

出　　处：《中华皮肤科杂志》2014年第10期715-718,共4页Chinese Journal of Dermatology

基　　金：国家自然科学基金（81171499）

摘　　要：目的 探讨副肿瘤性天疱疮的临床特点、治疗方法和预后.方法 对上海瑞金医院皮肤科病房收治的11例副肿瘤性天疱疮患者的临床资料进行回顾性分析,总结其临床特征.结果 11例副肿瘤性天疱疮患者中,10例有口唇、口腔黏膜的糜烂或溃疡.11例患者在出现皮损后经CT检查均发现内脏单发肿瘤,其中8例为Castleman病.6例患者出现阻塞性细支气管炎.所有患者术前曾应用糖皮质激素治疗无效.5例患者肿瘤切除后以小剂量糖皮质激素和沙利度胺联合治疗2～7个月皮肤及黏膜损害治愈,肺部症状缓解.结论 口腔黏膜糜烂或溃疡为副肿瘤性天疱疮最常见首发临床表现;Castleman病是最常见的伴发肿瘤;早期诊断和及时手术切除肿瘤是成功治疗的关键;术后糖皮质激素联合沙利度胺治疗有效.Objective To investigate the clinical features,treatment and prognosis of paraneoplastic pemphigus （PNP）.Methods A retrospective study was performed on 11 patients with PNP hospitalized in the Department of Dermatology,Ruijin Hospital.Clinical characteristics of these patients were analyzed.Results Of the 11 patients,10 had oral or labial erosions or ulcers,and 6 had obstructive bronchiolitis.Computed tomography （CT）showed solitary internal tumors in all the patients after appearance of skin lesions,and 8 of them were diagnosed with Castleman＇s disease.All the patients had been treated with corticosteroids before operation,but achieved no obvious improvement.After 2-7 months of postoperative treatment with low-dose prednisone and thalidomide,both cutaneous and mucosal lesions healed with the relief of pulmonary symptoms in 5 patients.Conclusions Oral erosions or ulcers appear to be the most common initial manifestation of PNP with Castleman＇s disease as the most frequent accompanying tumor.Early detection and timely resection of tumors are keys to successful treatment of PNP,and postoperative treatment with glucocorticoids and thalidomide proves to be effective for PNP.

关 键 词：天疱疮 副肿瘤性 回顾性研究 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]