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副肿瘤性天疱疮:慢性瘢痕性结膜炎的罕见病因。

Paraneoplastic pemphigus: an uncommon cause of chronic cicatrising conjunctivitis.

作者信息

Tam Patrick Mang Kwan, Cheng Lulu L, Young Alvin L, Lam Philip Tsze Ho

机构信息

Prince of Wales Hospital, Ophthalmology and Visual Sciences, 7th Floor, Block B Staff Quarters, Shatin, Hong Kong.

出版信息

BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.12.2008.1306. Epub 2009 Oct 14.

Abstract

We report a case of paraneoplastic pemphigus (PNP) as an uncommon but severe cause of cicatrising conjunctivitis. Initially diagnosed as drug eruptions, the patient's condition did not improve despite cessation of chemotherapy. Immunohistological confirmation of PNP has led to the use of combined oral prednisolone and intravenous immunoglobulin. Her ocular conditions stabilised with complete recovery of vision. PNP is a rare disease that can present with ocular involvement. Ophthalmologists should play an active role in monitoring and treatment of ocular surface complications such as symblepharon formation, severe dry eye and epithelial breakdown. Vigorous and prompt treatment is the key to successful prevention of irreversible and blinding complications. The atypical feature in this case is the presence of eosinophilic infiltration on histology that is a feature of allergic aetiologies rather than classical PNP.

摘要

我们报告一例副肿瘤性天疱疮(PNP),这是一种罕见但严重的瘢痕性结膜炎病因。该患者最初被诊断为药物疹,尽管停止了化疗,病情仍未改善。PNP的免疫组织学确诊促使采用口服泼尼松龙和静脉注射免疫球蛋白联合治疗。她的眼部状况稳定,视力完全恢复。PNP是一种可累及眼部的罕见疾病。眼科医生应在监测和治疗眼表并发症(如睑球粘连形成、严重干眼和上皮破损)方面发挥积极作用。积极及时的治疗是成功预防不可逆致盲并发症的关键。本例的非典型特征是组织学上存在嗜酸性粒细胞浸润,这是变应性病因而非经典PNP的特征。

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