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Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country

Epidermolysis Bullosa (EB) is a very rare genetic skin disorder associated with skin fragility. It results in blister formation on the skin. The purpose of this paper is to update the progress of a child with Dystrophic EB (DEB) who survived from infancy to preschool years and later died with recurr...

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Autores principales: Prabhakaran, Harshita, Arulappan, Judie, Elizabeth J, Sheeba, Cyril Vincent, Sophia
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9943966/
https://www.ncbi.nlm.nih.gov/pubmed/36845558
http://dx.doi.org/10.1177/2333794X231153507
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author Prabhakaran, Harshita
Arulappan, Judie
Elizabeth J, Sheeba
Cyril Vincent, Sophia
author_facet Prabhakaran, Harshita
Arulappan, Judie
Elizabeth J, Sheeba
Cyril Vincent, Sophia
author_sort Prabhakaran, Harshita
collection PubMed
description Epidermolysis Bullosa (EB) is a very rare genetic skin disorder associated with skin fragility. It results in blister formation on the skin. The purpose of this paper is to update the progress of a child with Dystrophic EB (DEB) who survived from infancy to preschool years and later died with recurrent skin blisters, bone marrow transplantation, and life support measures. A case analysis was done to evaluate the progress of the child. The mother of the child signed the written informed consent and granted permission to publish the details of the child with images and without disclosing the identifying information. The management of EB requires a multidisciplinary team approach. The care of the child should be aimed at protecting the child’s skin from injury, nutritional support, meticulous wound care, and management of complications as required. The prognosis varies from case to case.
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spelling pubmed-99439662023-02-23 Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country Prabhakaran, Harshita Arulappan, Judie Elizabeth J, Sheeba Cyril Vincent, Sophia Glob Pediatr Health Case Report Epidermolysis Bullosa (EB) is a very rare genetic skin disorder associated with skin fragility. It results in blister formation on the skin. The purpose of this paper is to update the progress of a child with Dystrophic EB (DEB) who survived from infancy to preschool years and later died with recurrent skin blisters, bone marrow transplantation, and life support measures. A case analysis was done to evaluate the progress of the child. The mother of the child signed the written informed consent and granted permission to publish the details of the child with images and without disclosing the identifying information. The management of EB requires a multidisciplinary team approach. The care of the child should be aimed at protecting the child’s skin from injury, nutritional support, meticulous wound care, and management of complications as required. The prognosis varies from case to case. SAGE Publications 2023-02-09 /pmc/articles/PMC9943966/ /pubmed/36845558 http://dx.doi.org/10.1177/2333794X231153507 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page(https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Case Report
Prabhakaran, Harshita
Arulappan, Judie
Elizabeth J, Sheeba
Cyril Vincent, Sophia
Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country
title Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country
title_full Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country
title_fullStr Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country
title_full_unstemmed Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country
title_short Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country
title_sort dystrophic epidermolysis bullosa in a preschooler in a middle eastern country
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9943966/
https://www.ncbi.nlm.nih.gov/pubmed/36845558
http://dx.doi.org/10.1177/2333794X231153507
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