Ichthyosis: A Road Model for Skin Research

The understanding of monogenetic disorders of cornification, including the group of diseases called ichthyoses, has expanded greatly in recent years. Studies of the aetiology of more than 50 types of ichthyosis have almost invariably uncovered errors in the biosynthesis of epidermal lipids or struct...

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Bibliographic Details
Main Authors: Anders Vahlquist, Hans Törmä
Format: Article
Language:English
Published: Medical Journals Sweden 2020-03-01
Series:Acta Dermato-Venereologica
Subjects:
Online Access: https://www.medicaljournals.se/acta/content/html/10.2340/00015555-3433
Description
Summary:The understanding of monogenetic disorders of cornification, including the group of diseases called ichthyoses, has expanded greatly in recent years. Studies of the aetiology of more than 50 types of ichthyosis have almost invariably uncovered errors in the biosynthesis of epidermal lipids or structural proteins essential for normal skin barrier function. The barrier abnormality per se may elicit epidermal inflammation, hyperproliferation and hyperkeratosis, potentially contributing to the patient’s skin symptoms. Despite this and other new knowledge about pathomechanisms, treatment of ichthyosis often remains unsatisfactory. This review highlights a series of approaches used to elucidate the pathobiology and clinical consequences of different types of ichthyosis, and related diseases with the ultimate goal of finding new and better treatments.
ISSN:0001-5555
1651-2057