The Ichthyoses—Pathogenesis and Prenatal Diagnosis: A Review of Recent Advances
作者:
Mary L. Williams,
期刊:
Pediatric Dermatology
(WILEY Available online 1983)
卷期:
Volume 1,
issue 1
页码: 1-24
ISSN:0736-8046
年代: 1983
DOI:10.1111/j.1525-1470.1983.tb01087.x
出版商: Blackwell Publishing Ltd
关键词: Ichthyosis;genodermatoses;stratum corneum;skin lipids;lipid metabolism
数据来源: WILEY
摘要:
Abstract:Disturbances in the process of normal cornification leading to pathologic scaling provide the pathophysiologic basis for the ichthyoses. These disturbances may result from either abnormalities in protein metabolism (keratinization) (i.e., the “bricks”) or in lipid metabolism (i.e., the “mortar”) (Fig. 1). The evidence linking the various ichthyoses to defects in protein or lipid metabolism have been reviewed. It is likely that future advances will lead not only to a more complete understanding of the pathogenesis of these disorders, but also will shed significant light on the normal stratum corneum functions of barrier formation and desquamation, as well as lead the way to more rational and effective therapies. In recent years, prenatal diagnosis has been successfully performed in several of the ichthyoses. It is likely that improvements in our ability to prenatally diagnose those disorders will advance hand‐in‐hand with further progress in unraveling their underl
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