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A case of a patient with severe epidermolysis bullosa surviving to adulthood

PURPOSE: The aim of this study was to evaluate the progression of a case of a patient with epidermolysis bullosa (EB) since early age who survived to adulthood, presenting with recurrent skin blisters and disfiguring scars and disabling musculoskeletal deformities. BACKGROUND: EB is a rare group of...

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Autores principales: Hubail, Amal R, Belkharoeva, Roza K, Tepluk, Natalya P, Grabovskaya, Olga V
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove Medical Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6241716/
https://www.ncbi.nlm.nih.gov/pubmed/30532579
http://dx.doi.org/10.2147/IJGM.S180464
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author Hubail, Amal R
Belkharoeva, Roza K
Tepluk, Natalya P
Grabovskaya, Olga V
author_facet Hubail, Amal R
Belkharoeva, Roza K
Tepluk, Natalya P
Grabovskaya, Olga V
author_sort Hubail, Amal R
collection PubMed
description PURPOSE: The aim of this study was to evaluate the progression of a case of a patient with epidermolysis bullosa (EB) since early age who survived to adulthood, presenting with recurrent skin blisters and disfiguring scars and disabling musculoskeletal deformities. BACKGROUND: EB is a rare group of inherited diseases that affect the skin fragility causing it to blister in response to even minor trauma. Established novel treatments are limited in the literature due to its rarity, and more research is needed to set a global management approach. Clinical manifestations range widely from localized to generalized blistering. METHODS: A rare case of EB surviving to adulthood despite the complications, which has been evaluated, treated during a relapse, and followed up. CONCLUSION: The described case is of considerable clinical interest due to its rarity and severity. Optimal management requires a multidisciplinary approach and revolves around the protection of the skin against slightest injury, use of careful wound care dressings, aggressive nutritional support, and early medical or surgical interventions if needed to manage any complications. Prognosis varies considerably depending on each case.
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spelling pubmed-62417162018-12-07 A case of a patient with severe epidermolysis bullosa surviving to adulthood Hubail, Amal R Belkharoeva, Roza K Tepluk, Natalya P Grabovskaya, Olga V Int J Gen Med Case Report PURPOSE: The aim of this study was to evaluate the progression of a case of a patient with epidermolysis bullosa (EB) since early age who survived to adulthood, presenting with recurrent skin blisters and disfiguring scars and disabling musculoskeletal deformities. BACKGROUND: EB is a rare group of inherited diseases that affect the skin fragility causing it to blister in response to even minor trauma. Established novel treatments are limited in the literature due to its rarity, and more research is needed to set a global management approach. Clinical manifestations range widely from localized to generalized blistering. METHODS: A rare case of EB surviving to adulthood despite the complications, which has been evaluated, treated during a relapse, and followed up. CONCLUSION: The described case is of considerable clinical interest due to its rarity and severity. Optimal management requires a multidisciplinary approach and revolves around the protection of the skin against slightest injury, use of careful wound care dressings, aggressive nutritional support, and early medical or surgical interventions if needed to manage any complications. Prognosis varies considerably depending on each case. Dove Medical Press 2018-11-15 /pmc/articles/PMC6241716/ /pubmed/30532579 http://dx.doi.org/10.2147/IJGM.S180464 Text en © 2018 Hubail et al. This work is published and licensed by Dove Medical Press Limited The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.
spellingShingle Case Report
Hubail, Amal R
Belkharoeva, Roza K
Tepluk, Natalya P
Grabovskaya, Olga V
A case of a patient with severe epidermolysis bullosa surviving to adulthood
title A case of a patient with severe epidermolysis bullosa surviving to adulthood
title_full A case of a patient with severe epidermolysis bullosa surviving to adulthood
title_fullStr A case of a patient with severe epidermolysis bullosa surviving to adulthood
title_full_unstemmed A case of a patient with severe epidermolysis bullosa surviving to adulthood
title_short A case of a patient with severe epidermolysis bullosa surviving to adulthood
title_sort case of a patient with severe epidermolysis bullosa surviving to adulthood
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6241716/
https://www.ncbi.nlm.nih.gov/pubmed/30532579
http://dx.doi.org/10.2147/IJGM.S180464
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