ENZYMATIC DISTINCTION BETWEEN 2 SUBGROUPS OF AUTOSOMAL RECESSIVE LAMELLAR ICHTHYOSIS

被引：19

作者：

BERGERS, M ^{[1
]}

TRAUPE, H ^{[1
]}

DUNNWALD, SC ^{[1
]}

MIER, PD ^{[1
]}

VANDOORENGREEBE, R ^{[1
]}

STEIJLEN, P ^{[1
]}

HAPPLE, R ^{[1
]}

机构：

[1] UNIV MUNSTER,DEPT DERMATOL,W-4400 MUNSTER,GERMANY

来源：

JOURNAL OF INVESTIGATIVE DERMATOLOGY | 1990年 / 94卷 / 04期

关键词：

D O I：

10.1111/1523-1747.ep12874475

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

It has been proposed that the autosomal recessive lamellar ichthyoses may be divided into two subgroups, the erythrodermic (EARLI) and non-erythrodermic (NEARLI) forms. We report measurements of the enzymes β-glucosidase, a recently described phospholipase, a short-chain carboxylesterase ("butyrase"), and a long-chain carboxylesterase ("palmitase") in aqueous extracts of scales from patients diagnosed according to clinical and micromorphologic criteria, and show that β-glucosidase and phospholipase tend to be lower in the EARLI group, whereas butyrase is relatively low in the NEARLI group. The internal ratio of either butyrase/glucosidase or butyrase/phospholipase yields a clear separation of the two subgroups, supporting the concept of heterogeneity in this group of diseases. © 1990.

引用

页码：407 / 412

页数：6

共 17 条

[1] ICHTHYOSIS CONGENITA TYPE-III - CLINICAL AND ULTRASTRUCTURAL CHARACTERISTICS AND DISTINCTION WITHIN THE HETEROGENEOUS ICHTHYOSIS CONGENITA GROUP [J].