伴有角膜沉积物的硬化性黏液水肿。

Scleromyxedema with corneal deposits.

作者信息

Goldin H M, Axelrod A J, Bronson D M, Torczynski E, Arroyave C M, Barsky S

机构信息

Department of Medicine, Cook County Hospital, Chicago, IL 60612.

出版信息

Ophthalmology. 1987 Oct;94(10):1334-8. doi: 10.1016/s0161-6420(87)80020-9.

DOI:10.1016/s0161-6420(87)80020-9

PMID:3684209

Abstract

Scleromyxedema (Arndt-Gottron syndrome) is a rare cutaneous disease in which hyaluronic acid is deposited in the dermis. The authors describe a patient with scleromyxedema and corneal deposits. A corneal biopsy demonstrated hyaluronic acid deposition in the corneal stroma and amyloid P component in Bowman's membrane. This is the first report of scleromyxedema involving the cornea. It is also the first report of amyloid P component deposition in the cornea occurring independent of corneal amyloid deposits.

摘要

硬化性黏液水肿（阿恩特-戈特龙综合征）是一种罕见的皮肤疾病，其中透明质酸沉积于真皮。作者描述了一名患有硬化性黏液水肿和角膜沉积物的患者。角膜活检显示透明质酸沉积于角膜基质，且淀粉样蛋白P成分沉积于Bowman膜。这是关于硬化性黏液水肿累及角膜的首例报告。也是角膜中淀粉样蛋白P成分独立于角膜淀粉样沉积物发生沉积的首例报告。