Commentary: New advances in the development of therapies for treating inherited skin fragility disorders
نویسنده
چکیده
Inherited skin fragility disorders comprise a group of disorders, mainly designated as epidermolysis bullosa (EB), that are characterised by mechanical induced blistering and erosions of the skin and mucous membranes caused by mutations of gene coding for protein components of the dermal–epidermal junction zone. Patients with EB experience various degrees of recurrent skin blistering, widespread bullae, and erosions that characteristically heal with exuberant scarring and milia formation or lead to impaired non-healing wounds and the development of aggressive skin cancers. Advances in understanding the pathogenesis of EB in the last decade have led to the identification of several candidate genes and proteins and the development of several therapeutic strategies currently progressing to pre-clinical and clinical stages. This review will focus on the recent advances in the development of these new therapies for treating inherited skin fragility disorders (with particular focus on recessive dystrophic epidermolysis bullosa), which hold great promise for improving the quality of life for patients suffering from EB.
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