副肿瘤性天疱疮致眼部病变的病例回顾分析  

Retrospective analysis of eye changes of paraneoplastic pemphigus

作　　者：宫海军[1] 蓝育青[1] 王梅[1] 胡玉新[1] 

机构地区：[1]中山大学孙逸仙纪念医院眼科,广州510120

出　　处：《中国眼耳鼻喉科杂志》2017年第6期392-394,共3页Chinese Journal of Ophthalmology and Otorhinolaryngology

摘　　要：目的探讨副肿瘤性天疱疮(PNP)眼部病变的临床特点并分析其误诊、误治原因。方法回顾系列病例研究。回顾性分析2008年4月~2016年7月于本院皮肤科确诊,同时经眼科会诊的6例PNP患者(12眼)的病史、全身情况、病理学结果、眼科症状及体征,分析其误诊、误治原因。结果经临床表现、影像学检查,以及皮肤、黏膜活检,4例诊断为Castleman病伴发PNP,1例诊断为淋巴瘤伴发PNP,1例为滤泡树突细胞肉瘤合并Castleman病伴发PNP。所有患者眼部早期均为慢性结膜炎表现,3例出现了眼睑溃疡和结膜瘢痕,1例出现了角膜溃疡。所有病例确诊前都按慢性结膜炎长期滴用抗生素治疗,但症状无缓解。确诊PNP后,4例患者经全身肿块切除及合理治疗后眼部瘢痕逐渐愈合,另外2例患者因全身病情未能控制而死亡。结论PNP伴发眼部病变早期表现为慢性结膜炎,极易误诊,可引起严重的眼表瘢痕化。提高对PNP的认识,及时规范给予眼部干预措施是保存患者视力的重要手段。Objective To analyze the ocular clinical characteristics and the causes of misdiagnosis of paraneoplastic pemphigns （PNP）. Methods It was a retrospective series case study. Six patients referred for the evaluation of possible PNP from April 2008 to July 2016 in Departments of Ophthalmology and Dermatology of Sun Yat- sen Memorial Hospital of Sun Yat-sen University. The history, pathology, ocular and systematic clinical characterization of 6 cases（ 12 eyes） were analyzed to find the causes of misdiagnosis and to explore the reasonable treatment. Results All cases complained recurrent mucous and skin symptoms. Four cases were diagnosed as Castleman disease, one case was diagnosed as Castleman disease combined with Follicular dendritic cell sarcoma, and one case was diagnosed as lymphoma. All cases presented chronic conjunctivitis during the early stage of the disease, three cases gradually appeared eyelid ulcer and conjunctival scarfing, one case gradually appeared keratohelcosis. All cases were treated with antibiotic eye drops before PNP diagnosis. In four cases, scar healing and inflammation was controlled by neoplasms resection combined with systematic and ocular therapy. Cicatrization of two cases still progressed and failed to control, dead finally. Conclusions Ocular manifestation of PNP can mimic chronic conjunctivitis during the early stage. It is easy to be misdiagnosed. It may lead to severe ocular surface scars. Ophthalmologists need to increase awareness of PNP. Timely and standardized treatment is the key to preservation of vision.

关 键 词：副肿瘤性天疱疮 CASTLEMAN病 眼部病变 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]