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硬化性黏液水肿的角膜表现

Corneal manifestations of scleromyxedema.

作者信息

Pusateri T J, Margo C E, Groden L R

出版信息

Ophthalmology. 1987 May;94(5):510-3. doi: 10.1016/s0161-6420(87)33428-1.

Abstract

Bilateral superficial corneal deposits and scleromyxedema, an uncommon dermatosis caused by the accumulation of acid mucopolysaccharide in the skin often associated with a benign monoclonal gammopathy developed in a 71-year-old man. A biopsy specimen of the cornea showed that the deposits stained strongly positive for IgG and lambda chains. Ultrastructurally, the deposits consisted of amorphous granular material. Scleromyxedema should be considered in the differential diagnosis of noninflammatory superficial keratopathies associated with benign gammopathies.

摘要

一名71岁男性出现双侧浅表角膜沉积物和硬化性黏液水肿,后者是一种因酸性黏多糖在皮肤积聚所致的罕见皮肤病,常与良性单克隆丙种球蛋白病相关。角膜活检标本显示沉积物对IgG和λ链染色呈强阳性。超微结构上,沉积物由无定形颗粒物质组成。在与良性丙种球蛋白病相关的非炎症性浅表角膜病变的鉴别诊断中应考虑硬化性黏液水肿。

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