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脱屑性内皮病变。一种早期的虹膜角膜内皮综合征?

Desquamating endotheliopathy. An incipient iridocorneal endothelial syndrome?

作者信息

Stock E L, Roth S I, Morimoto D

机构信息

Cornea and External Eye Disease Laboratory, Veterans Administration Lakeside Medical Center, Chicago, IL.

出版信息

Arch Ophthalmol. 1987 Oct;105(10):1378-81. doi: 10.1001/archopht.1987.01060100080031.

DOI:10.1001/archopht.1987.01060100080031
PMID:3310985
Abstract

Unilateral, noninfectious, nontraumatic corneal endotheliopathy was noted in a 34-year-old man who had had blurred vision for five years without evidence of iridic disease or glaucoma. Ultrastructural studies demonstrated focal necrosis of the corneal endothelial cells, with desquamation of the cells into the anterior chamber. The corneal endothelium appeared to expand beneath the dying endothelial cells, indicating reendothelialization of the cornea. There was no epithelialization of the endothelium, as evidenced by the lack of keratin production or desmosome formation. Descemet's membrane was thickened with edema, a posterior collagenous layer, and fibrous, long-spacing collagen. These alterations in Descemet's membrane were similar to those described for other corneal dystrophies. It is proposed that this unilateral desquamating endotheliopathy represents an incipient form or a forme fruste of the iridocorneal endothelial syndrome.

摘要

一名34岁男性被发现患有单侧、非感染性、非创伤性角膜内皮病变,他有五年视力模糊史,无虹膜疾病或青光眼迹象。超微结构研究显示角膜内皮细胞局灶性坏死,细胞脱屑进入前房。角膜内皮似乎在濒死的内皮细胞下方扩张,表明角膜有内皮再生。内皮未出现上皮化生,这可通过缺乏角蛋白生成或桥粒形成得到证明。Descemet膜因水肿、后胶原层以及纤维性长间距胶原而增厚。Descemet膜的这些改变与其他角膜营养不良所描述的改变相似。有人提出这种单侧脱屑性内皮病变代表虹膜角膜内皮综合征的初期形式或顿挫型。

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