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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Dove Medical Press
2018
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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. |
format | Online Article Text |
id | pubmed-6241716 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | Dove Medical Press |
record_format | MEDLINE/PubMed |
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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