Therapies for inherited skin fragility disorders.

Abstract:

:Inherited skin fragility comprises disorders characterized by mechanical induced blistering and erosions within the skin and mucosal membranes as a consequence of mutations in genes encoding proteins involved in intra-epidermal or dermal-epidermal adhesion. As the molecular pathology is largely known, it is a prototype group of disorders for which numerous experimental treatments have been developed. However, it became clear that single therapeutic strategies will not be able to address all molecular and clinical aspects. Significant progress has been achieved in gene, cell and protein therapies. Although the way towards clinical application seems obvious, major challenges must be addressed before these therapies become largely accessible. Until curative treatments will become available, alternative strategies which aim at increasing protein stability, amending apoptosis, inflammation and scarring may alleviate skin fragility and prevent or delay the onset of complications.

journal_name

Exp Dermatol

journal_title

Experimental dermatology

authors

Has C,Kiritsi D

doi

10.1111/exd.12666

subject

Has Abstract

pub_date

2015-05-01 00:00:00

pages

325-31

issue

5

eissn

0906-6705

issn

1600-0625

journal_volume

24

pub_type

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