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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Bibliographic Details
Published inGlobal pediatric health Vol. 10; p. 2333794X231153507
Main Authors Prabhakaran, Harshita, Arulappan, Judie, Elizabeth J, Sheeba, Cyril Vincent, Sophia
Format Journal Article
LanguageEnglish
Published Los Angeles, CA SAGE Publications 01.01.2023
Sage Publications Ltd
SAGE Publishing
Subjects
Bone marrow
Case Report
Case reports
Genetic disorders
Preschool children
Skin diseases
childhood
epidermolysis bullosa
blistering skin diseases
survival
skin fragility
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Summary: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.
ISSN:2333-794X
2333-794X
DOI:10.1177/2333794X231153507