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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| Published in | Global pediatric health Vol. 10; p. 2333794X231153507 |
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| Main Authors | , , , |
| Format | Journal Article |
| Language | English |
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Los Angeles, CA
SAGE Publications
01.01.2023
Sage Publications Ltd SAGE Publishing |
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| Abstract | 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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| AbstractList | 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. 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. |
| Author | Prabhakaran, Harshita Arulappan, Judie Elizabeth J, Sheeba Cyril Vincent, Sophia |
| AuthorAffiliation | 1 Department of Maternal and Child Health, College of Nursing, Sultan Qaboos University, Al Khoudh, Muscat, Sultanate of Oman |
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| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/36845558$$D View this record in MEDLINE/PubMed |
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| Cites_doi | 10.1038/nature24487 10.1002/humu.21551 10.1186/1750-1172-9-76 10.1073/pnas.0706486104 10.1016/j.clindermatol.2011.03.012 10.1186/s13023-019-1086-5 10.1111/pde.12409 10.3109/09546634.2014.915002 10.1038/s41572-020-0210-0 10.1016/j.jaad.2012.01.016 10.12968/bjon.2016.25.8.428 10.1016/bs.ctm.2015.05.003 10.1016/j.jaad.2013.09.041 10.1067/mjd.2002.119673 10.1016/j.jaad.2008.02.004 10.1016/j.jaad.2015.04.015 10.1177/1049732308315110 |
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| Copyright | The Author(s) 2023 The Author(s) 2023. The Author(s) 2023. This work is licensed under the Creative Commons Attribution – Non-Commercial License https://creativecommons.org/licenses/by-nc/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License. The Author(s) 2023 2023 SAGE Publications |
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| Keywords | childhood epidermolysis bullosa blistering skin diseases survival skin fragility |
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Their Caregivers: DEBRA Italy. Brief Presentation of Results year: 2016 ident: bibr20-2333794X231153507 contributor: fullname: Pilo C. – volume: 8 start-page: 6 issue: 41 year: 2015 ident: bibr16-2333794X231153507 publication-title: J Clin Aesthet Dermatol contributor: fullname: Soro L – ident: bibr6-2333794X231153507 doi: 10.1016/j.jaad.2008.02.004 – ident: bibr8-2333794X231153507 doi: 10.1016/j.jaad.2015.04.015 – ident: bibr18-2333794X231153507 doi: 10.1177/1049732308315110 – ident: bibr22-2333794X231153507 |
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| Snippet | 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... 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... |
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| StartPage | 2333794X231153507 |
| SubjectTerms | Bone marrow Case Report Case reports Genetic disorders Preschool children Skin diseases |
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| Title | Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country |
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