Cargando…

Management of Recessive Dystrophic Epidermolysis Bullosa in a Newborn with Porcine-derived Extracellular Matrix

Epidermolysis bullosa is a debilitating dermatologic disorder affecting the adhesive capability between the epidermis and dermis. The severe recessive dystrophic variant is caused by mutations in COL7A1, the gene encoding type VII collagen which is the major structural protein of the anchoring fibri...

Descripción completa

Detalles Bibliográficos
Autores principales: Le, Nicole K., Billington, Alicia, Harrington, Michael, Seminario-Vidal, Lucia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6908358/
https://www.ncbi.nlm.nih.gov/pubmed/31942309
http://dx.doi.org/10.1097/GOX.0000000000002519
Descripción
Sumario:Epidermolysis bullosa is a debilitating dermatologic disorder affecting the adhesive capability between the epidermis and dermis. The severe recessive dystrophic variant is caused by mutations in COL7A1, the gene encoding type VII collagen which is the major structural protein of the anchoring fibrils linking these 2 skin layers.1 The management of recessive dystrophic epidermolysis bullosa (RDEB) remains complex with no curative therapy. We present herein the novel use of a porcinederived extracellular matrix dressing to effectively treat extensive erosions in a newborn.