PROTEIN-S DEFICIENCY IN LUPUS-ERYTHEMATOSUS SECONDARY TO HEREDITARY ANGIO-EDEMA

被引：12

作者：

PERKINS, W ^{[1
]}

STABLES, GI ^{[1
]}

LEVER, RS ^{[1
]}

机构：

[1] WESTERN INFIRM & ASSOCIATED HOSP,DEPT DERMATOL,GLASGOW G11 6NT,SCOTLAND

来源：

BRITISH JOURNAL OF DERMATOLOGY | 1994年 / 130卷 / 03期

关键词：

D O I：

10.1111/j.1365-2133.1994.tb02937.x

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

We report a child with lupus erythematosus secondary to type 2 hereditary angio-oedema (HAE). Reduced levels of free protein S were found. Total protein S, and C4 binding protein (C4bp) were normal, and there was no evidence of anticardiolipin antibodies or lupus anticoagulant. We postulate that reduced levels of C4, secondary to C1 inhibitor deficiency, resulted in increased binding of protein S to C4bp, leading to reduced levels of free protein S and thus functional deficiency of protein S.

引用

页码：381 / 384

页数：4

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