Pathogenetic Therapy of Epidermolysis Bullosa: Current State and Prospectsстатья
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Дата последнего поиска статьи во внешних источниках: 7 июля 2021 г.
Аннотация:Epidermolysis bullosa is a severe hereditary disease caused by mutations in genes encodingcutaneous basement membrane proteins. These mutations lead to dermal-epidermal junctionfailure and, as a result, to disturbances in the morphological integrity of the skin. Clinically,it manifests in the formation of blisters on the skin or mucosa that in some cases can turninto non-healing chronic wounds, which not only impairs patient’s quality of life, but also isa live-threatening condition. Now, the main approaches in the treatment of epidermolysis bullosa are symptomatic therapy and palliative care, though they are little efective and are aimedat reducing the pain, but not to complete recovery. In light of this, the development of newtreatment approaches aimed at correction of genetic defects is in progress. Various methodsbased on genetic engineering technologies, transplantation of autologous skin cells, progenitor skin cells, as well as hematopoietic and mesenchymal stem cells are studied. This reviewanalyzes the pathogenetic methods developed for epidermolysis bullosa treatment based onthe latest achievements of molecular genetics and cellular technologies, and discusses theprospects for the use of these technologies for the therapy of epidermolysis bullosa.