腹部局限性Castleman病副肿瘤性天疱疮八例临床分析

Treatment of paraneoplastic pemphigus with Castleman′s disease

目的 探讨腹部局限性Castleman病副肿瘤性天疱疮的临床表现和临床治疗方法。方法 对我院收治的 8例伴有腹部局限性Castleman病的副肿瘤性天疱疮患者的临床资料进行分析 ,探讨该病的临床和病理特征 ,总结其外科治疗效果。结果 8例患者均有典型天疱疮皮肤损害 ,伴有难治性口唇黏膜溃疡、糜烂 ;病理改变表现为表皮内疱、基底细胞液化变性 ,表皮内散在坏死的角质形成细胞及真皮浅层以淋巴细胞浸润为主。 8例患者CT检查均发现内脏单发肿瘤。 5例出现阻塞性细支气管炎。所有患者常规激素治疗无效 ,肿瘤切除后药物治疗 1～ 2个月皮肤及黏膜损害治愈 ,肺部病变缓解 ,切除的肿瘤位于腹膜后 ,病理改变符合Castleman病。结论 腹部局限性Castleman病副肿瘤性天疱疮是一罕见疾病 ,发现和切除合并的内脏肿瘤是治疗的关键。

Objective To discuss the clinical findings and treatment of paraneoplastic pemphigus(PNP)with Castleman′s disease Methods To investigate the clinical,histopathologic and CT findings of 8 cases paraneoplastic pemphigus with Castleman′s disease Results All of 8 patients were diagnosed PNP first and were found Castleman′s tumor incidently during routine examination All 8 cases showed severe erosion or ulcer of the oral mucosa with various skin lesions Histopathologically,there were intraepidermal acantholytic vesicle,basal cell liquefication,necrotic keratinocytes in the epidermis and lymphocyte infiltration in the upper dermis CT scan appeared solitary mass in these patients Some of them were attacked by bronchiolitis obliterans All 8 patients were failed by use of predisone Obvious relief of PNP and pulmonary lesion occurred after tumor was rescted Conclusions Paraneoplastic pemphigus with Castleman′s disease is a rare disease The key step is to find and resect the tumor in abdomen CT scan should be used to detect the tumor in patients with PNP,especially,when predisone was failed in treatment