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眼部瘢痕性类天疱疮

Author
Stephen Foster, MD
Section Editor
John J Zone, MD
Deputy Editor
Abena O Ofori, MD
Translators
林麟, 主任医师

引言

黏膜类天疱疮是一组异质性慢性炎症性水疱性疾病,可累及口、眼、咽、喉、生殖器或肛门黏膜。这组疾病共有的特征性病理改变是在上皮基底膜区出现IgG、IgA或C3的线状沉积。术语眼部瘢痕性类天疱疮(ocular cicatricial pemphigoid, OCP)是指临床上表现为慢性瘢痕性(瘢痕形成)结膜炎的黏膜类天疱疮。OCP也可累及其他的黏膜部位和非黏膜皮肤。

如果OCP未经治疗，最终会导致严重的结膜瘢痕形成和视力丧失。因此，理想的处理是早期诊断并立即开始治疗。OCP的治疗包括使用系统性免疫调节药物来抑制结膜炎症。认真的眼部保健也很重要，它可以最大限度地减轻慢性炎症带来的后果。

本文将总结OCP的发病机制、临床表现、诊断和治疗。其他类型的类天疱疮将单独讨论。(参见“Clinical features and diagnosis of bullous pemphigoid and mucous membrane pemphigoid”)

流行病学

据估计，在眼科患者中，OCP的发病率为1/46,000-1/8000[1-4]。并未发现该病有地域或种族的好发倾向。

OCP主要发生于老年患者，诊断的平均年龄在60-70岁[5-7]。然而，由于OCP早期表现为非特异性结膜炎症，所以在某些患者中该病可能在诊断之前已存在多年。

                          

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Literature review current through: 2017-06 . | This topic last updated: 2015-08-05.
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