7例副肿瘤性天疱疮临床分析

Retrospective Analysis of Seven Patients with Paraneoplastic Pemphigus

目的明确副肿瘤性天疱疮的临床特点、治疗方法及预后。方法回顾分析我院2008年到2013年间收治的副肿瘤性天疱疮患者7例,分析其临床表现、伴随的肿瘤及组织病理学特点和实验室检查、治疗方法及预后。结果所有患者均有严重的黏膜损害,以口腔黏膜损害为突出表现,皮损以多形红斑、扁平苔藓样皮损和大疱最常见,最常见的特征性组织病理学为界面皮炎和凋亡的角质形成细胞。7例患者均为内脏单发肿瘤,其中以Castleman病最常见(n=6),胸腺瘤(n=1)。其中3例死亡,1例手术后死于肺部感染,1例因自行停药而死亡,1例手术前死于败血症和严重复杂的多部位感染。手术后存活4例,最长已达5年以上,出现阻塞性细支气管炎仍未完全缓解;1例于手术后2年痊愈停药;另2例仍在口服泼尼松治疗。结论 Castleman病是该组患者最常见的伴发肿瘤,及早发现并切除肿瘤是关键措施,手术前后的正确处理可提高其生存率。

Objective To determine the clinical findings, treatment and clinical outcomes of paraneoplastic pemphigus （PNP）, a rare, life--threatening, autoimmune blistering disease associated with neoplasia. Methods Seven patients with PNP that were diagnosed between 2008 and 2013 were retrospectively analyzed. Analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory fingdings were performed . Results All of the patients had severe mucosal involvement. Erythema multiforme, lichen planus-like eruptions and bullous lesions were commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinoeytes. CT scan appeared solitary mass in these patient, with Castleman＇ s disease（n=6） as the most frequent. Seven patients were followed for 5-60 months. Three patients died dut to postoperation infection （n=l）, serious infections in multiple sites before operation （n =1 ） and drug discontinuance postoperation （n=l）. Conclusion Castleman＇ s disease is the most frequent neoplasm in these patients. Early diagnosis and prompt removal of the tumor are key to the management of this disease, the appropriate treatment of postoperation can improve the prognosis.