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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 investigate the clinical manifestations and clinical treatment of Castleman’s disease paraneoplastic pemphigus with localized limitations. Methods The clinical data of 8 paraneoplastic pemphigoid patients with Castleman’s disease localized in the abdomen were analyzed in our hospital to investigate the clinical and pathological features of the disease and to summarize the surgical treatment results. Results All the 8 patients had typical pemphigus skin lesions accompanied by refractory lip mucosal ulceration and erosion. The pathological changes were intrasternal epidermis, liquefaction of basal cells, keratinocytes scattered in the epidermis and superficial dermis Mainly lymphocyte infiltration. All 8 patients had visceral solitary tumors on CT examination. Five patients developed obstructive bronchiolitis. All patients with conventional hormone therapy ineffective, 1 to 2 months after tumor resection drug treatment of skin and mucosal lesions were cured, alleviated lung disease, resection of the tumor located in the retroperitoneal, pathological changes consistent with Castleman’s disease. Conclusions Abdominal limitations of Castleman’s disease Paraneoplastic pemphigus is a rare disease found and resected in combination with visceral tumors is the key to treatment.