副肿瘤性天疱疮并发闭塞性细支气管炎1例报道并文献复习  

Paraneoplastic pemphigus complicated with bronchiolitis obliterans:a case report and literature review

作　　者：李敏[1] 黄孝庆[1] 陈方淳[1] LI Min;HUANG Xiaoqing;CHEN Fangchun(College of Stomatology,Chongqing Medical University/Chongqing Key Laboratory of Oral Diseases and Biomedical Sciences/Chongqing Municipal Key Laboratory of Oral Biomedical Engineering of Higher Education,Chongqing 401120,China)

机构地区：[1]重庆医科大学附属口腔医院/口腔疾病与生物医学重庆市重点实验室/重庆市高校市级口腔生物医学工程重点实验室,重庆401120

出　　处：《重庆医学》2020年第16期2719-2722,共4页Chongqing medicine

基　　金：重庆市科卫联合中医药科研项目(2019ZY023226)。

摘　　要：目的探讨副肿瘤性天疱疮(PNP)并发闭塞性细支气管炎(BO)的临床特点和诊治方法。方法回顾性分析1例Castleman病术后PNP并发BO患者的诊治经过,结合国内外文献报道的相关病例特征进行分析。结果患者盆腔肿块(最后诊断为Castleman病)术后出现难治性的口腔糜烂,使用糖皮质激素及免疫抑制剂效果欠佳,后出现呼吸困难,最终诊断为PNP并发BO(Castleman病术后)。文献复习发现,Castleman病患者中有超过一半(16例,69.6%)的病灶位于腹腔(腹膜后或腹盆腔),纵隔病变和外周病变少见。Castleman病本身并不造成患者的自觉不适,当诱发PNP时,才会出现特有的症状和体征。PNP出现1~8个月后常并发BO,即使并发肿瘤被切除后,部分患者仍可出现不可逆的呼吸困难甚至Ⅱ型呼吸衰竭,一旦出现呼吸系统受累,即使手术切除原发病灶、应用较强的免疫抑制治疗,仍无法逆转肺内病变的进展。结论BO是PNP最严重的并发症,应尽早诊断和规范治疗。Objective To investigate the clinical characteristics,diagnosis and treatment methods of paraneoplastic pemphigus(PNP)combined with bronchiolitis obliterans(BO).Methods A case of PNP combined with BO after Castleman′s disease operation was analyzed retrospectively,combined with the characteristics of related cases reported in domestic and foreign literature.Results The patients with pelvic mass(the final diagnosis was Castleman′s disease)developed refractory oral erosion,poor effect of glucocorticoids and immunosuppressive agents,then dyspnea,and finally diagnosed as PNP with BO(Castleman′s disease after operation).Literature review found that there were more than half Castleman′s disease patients(16 cases,69.6%)were located in the abdominal cavity(retroperitoneal or pelvic),while mediastinal and peripheral lesions were rare.Patients with Castleman′s disease did not feel discomfort,while PNP was induced,it would have special symptoms and signs.BO often occurred at one to eight months after PNP,even the tumor was removed,some patients still had irreversible dyspnea or typeⅡrespiratory failure.The research showed that once the respiratory system was involved,even if the primary focus was removed by surgery or the immunosuppressive therapy was applied,the progress of pulmonary lesions could not be reversed.Conclusion BO is the most serious complication of PNP,early diagnosis and standardized treatment should be carried out.

关 键 词：副肿瘤性天疱疮 闭塞性细支气管炎 巨淋巴结增生 手术后并发症 病例报告 

分 类 号：R781.59[医药卫生—口腔医学]