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瘢痕性类天疱疮:病例报告。

Ocular cicatricial pemphigoid: case report.

作者信息

Louie T D

机构信息

Albuquerque Veterans Administration Medical Center, New Mexico, USA.

出版信息

J Am Optom Assoc. 1998 Mar;69(3):153-60.

PMID:9553330
Abstract

BACKGROUND

The main characteristic of ocular cicatricial pemphigoid is subepithelial conjunctival fibrosis, which, if progressive, can lead to conjunctival shrinkage and symblepharon formation. Other characteristics include entropion, trichiasis, dry eye, cornea ulceration, neovascularization, and keratinization. Ultimately, blindness may occur. Cicatricial pemphigoid may also affect the skin and mucous membranes, including the nose, oral cavity, pharynx, esophagus, anus, and vagina. Treatment involves systemic immunosuppressants and corticosteroids to suppress active inflammation.

CASE REPORT

A 69-year-old man had symblephara, conjunctival shortening, and extraocular manifestations, and was diagnosed with ocular cicatricial pemphigoid on the basis of clinical appearance and immunofluorescent studies. Treatment included oral Dapsone, prednisone, and azathioprine to stabilize the condition.

CONCLUSION

Close monitoring of ocular cicatricial pemphigoid is imperative for ocular manifestations and for extraocular complications occurring with this progressive autoimmune disease and its treatment.

摘要

背景

瘢痕性类天疱疮的主要特征是结膜上皮下纤维化,若病情进展,可导致结膜收缩和睑球粘连形成。其他特征包括睑内翻、倒睫、干眼、角膜溃疡、新生血管形成和角化。最终可能导致失明。瘢痕性类天疱疮也可累及皮肤和黏膜,包括鼻、口腔、咽、食管、肛门和阴道。治疗包括使用全身免疫抑制剂和皮质类固醇来抑制活动性炎症。

病例报告

一名69岁男性有睑球粘连、结膜缩短和眼外表现,根据临床表现和免疫荧光检查诊断为瘢痕性类天疱疮。治疗包括口服氨苯砜、泼尼松和硫唑嘌呤以稳定病情。

结论

对于瘢痕性类天疱疮这种进行性自身免疫性疾病及其治疗过程中出现的眼部表现和眼外并发症,必须进行密切监测。

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