Review of the Latest Methods of Epidermolysis Bullosa and Other Chronic Wounds Treatment Including BIOOPA Dressing

Abstract

Epidermolysis bullosa (EB) is a hereditary genetic skin disorder, classified as a type of genodermatosis, which causes severe, chronic skin blisters associated with painful and potentially life-threatening complications. Currently, there is no effective therapy or cure for EB. However, over the past decade, there have been several important advances in treatment methods, which are now approaching clinical application, including gene therapy, protein replacement therapy, cell therapy (allogeneic fibroblasts, mesenchymal stromal cells), bone marrow stem cell transplant, culture/vaccination of revertant mosaic keratinocytes, gene editing/engineering, and the clinical application of inducible pluripotent stem cells. Tissue engineering scientists are developing materials that mimic the structure and natural healing process to promote skin reconstruction in the event of an incurable injury. Although a cure for EB remains elusive, recent data from animal models and preliminary human clinical trials have raised the expectations of patients, clinicians, and researchers, where modifying the disease and improving patients' quality of life are now considered attainable goals. In addition, the lessons learned from the treatment of EB may improve the treatment of other genetic diseases.

Care problems of a newborn with epidermolysis bullosa – a case study

“…Choroba jest genetycznie uwarunkowaną rzadką dermatozą skórną. Powstaje w wyniku upośledzenia w połączeniu warstw skóry właściwej i naskórka [1][2][3], na skutek czego zmniejszona jest wytrzymałość skóry na obciążenia mechaniczne [4,5]. Nawet najmniejszy ucisk lub otarcie skutkuje powstaniem ubytków skóry i niegojących się pęcherzy wypełnionych surowiczym płynem, które w następstwie z powodu kruchości tkanek mogą tworzyć rozległe rany i nadżerki [6][7][8].…”

Epidermolysis Bullosa: A Review of the Tissue-Engineered Skin Substitutes Used to Treat Wounds

“…As these cell types express alternative signalling pathways to skin cells, their addition may also enable the generation of anatomic skin structures [ 78 ]. One group assessed a biological dressing composed of ~30 million Wharton’s jelly-derived mesenchymal stem cells seeded onto an acellular human skin matrix of allogeneic origin to treat an EB wound [ 79 ]. An assessment 30 days after transplantation was promising and revealed that the dressing had been infiltrated by host cells and neovascularisation had occurred [ 79 ].…”

“…One group assessed a biological dressing composed of ~30 million Wharton’s jelly-derived mesenchymal stem cells seeded onto an acellular human skin matrix of allogeneic origin to treat an EB wound [ 79 ]. An assessment 30 days after transplantation was promising and revealed that the dressing had been infiltrated by host cells and neovascularisation had occurred [ 79 ]. Finally, as immune cells such as macrophages and neutrophils play an important role as inflammatory effectors during wound healing, their addition could enhance the regeneration potential of future TESSs [ 6 ].…”