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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...

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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
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author Le, Nicole K.
Billington, Alicia
Harrington, Michael
Seminario-Vidal, Lucia
author_facet Le, Nicole K.
Billington, Alicia
Harrington, Michael
Seminario-Vidal, Lucia
author_sort Le, Nicole K.
collection PubMed
description 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.
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spelling pubmed-69083582020-01-15 Management of Recessive Dystrophic Epidermolysis Bullosa in a Newborn with Porcine-derived Extracellular Matrix Le, Nicole K. Billington, Alicia Harrington, Michael Seminario-Vidal, Lucia Plast Reconstr Surg Glob Open Case Report 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. Wolters Kluwer Health 2019-11-12 /pmc/articles/PMC6908358/ /pubmed/31942309 http://dx.doi.org/10.1097/GOX.0000000000002519 Text en Copyright © 2019 The Authors. Published by Wolters Kluwer Health, Inc. on behalf of The American Society of Plastic Surgeons. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (http://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Case Report
Le, Nicole K.
Billington, Alicia
Harrington, Michael
Seminario-Vidal, Lucia
Management of Recessive Dystrophic Epidermolysis Bullosa in a Newborn with Porcine-derived Extracellular Matrix
title Management of Recessive Dystrophic Epidermolysis Bullosa in a Newborn with Porcine-derived Extracellular Matrix
title_full Management of Recessive Dystrophic Epidermolysis Bullosa in a Newborn with Porcine-derived Extracellular Matrix
title_fullStr Management of Recessive Dystrophic Epidermolysis Bullosa in a Newborn with Porcine-derived Extracellular Matrix
title_full_unstemmed Management of Recessive Dystrophic Epidermolysis Bullosa in a Newborn with Porcine-derived Extracellular Matrix
title_short Management of Recessive Dystrophic Epidermolysis Bullosa in a Newborn with Porcine-derived Extracellular Matrix
title_sort management of recessive dystrophic epidermolysis bullosa in a newborn with porcine-derived extracellular matrix
topic Case Report
url 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
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