副肿瘤性天疱疮6例临床分析

Retrospective Analysis of Six Patients with Paraneoplastic Pemphigus

目的探讨副肿瘤性天疱疮的临床特点、治疗与转归。方法回顾性分析本院诊治的6例副肿瘤性天疱疮患者的临床表现、组织病理学和免疫学特点及合并肿瘤情况、治疗方法与转归。结果所有患者均有黏膜损害,以口腔黏膜损害最突出,其中5例以口腔黏膜损害为首发症状,皮损以水疱、表皮剥脱、多形红斑、扁平苔藓样皮损常见,主要特征性的组织病理学表现为表皮内棘层松解和角质形成细胞坏死,其次为苔藓样皮炎病理征象。6例患者均合并肿瘤。6例患者除予以肿瘤针对性治疗外,均予以糖皮质激素治疗,其中4例同时联合大剂量静脉用免疫球蛋白(IVIG)治疗,最终5例患者死亡,死因分别为呼吸衰竭、肺部感染、肿瘤恶化等。结论副肿瘤性天疱疮发病机制复杂,临床皮损多样,预后差,及早诊断并同时针对肿瘤及副肿瘤天疱疮治疗,对预后做好适当管理,可提高其生存率。

Objective To investigate the clinical characteristics,treatment and outcome of paraneoplastic pemphigus(PNP).Methods Retrospectively analysis the clinical manifestations,histopathological and immunological features,concomitant tumor,treatment and outcome of 6 patients with PNP was conducted.Results All of the patients had mucosal involvement,especially oral mucosa damage,oral mucosa damage was the first symptom in 5 cases.Blisters,exfoliation,erythema multiforme,lichen planus-like eruptions were commonly skin rashes.The main histopathological features were intraepidermal acantholysis and keratinocyte necrosis,followed by lichenoid dermatitis.All 6 patients were complicated with tumor,and visceral tumor was the most common.In addition to tumor-specific therapy,all the 6 patients were treated with glucocorticoid,and 4 of them were simultaneously treated with high-dose intravenous immunoglobulin.Finally,5 patients died from respiratory failure,pulmonary infection and tumor deterioration,etc.Conclusion Paraneoplastic pemphigus has complex pathogenesis,various clinical lesions and poor survival rate,early diagnosis,simultaneous treatment of tumor and PNP and proper prognosis management can improve the survival rate.