Dystrophic Epidermolysis Bullosa in a Preschooler in a Middle Eastern Country

Author:

Prabhakaran Harshita1,Arulappan Judie1ORCID,Elizabeth J Sheeba1,Cyril Vincent Sophia1

Affiliation:

1. Department of Maternal and Child Health, College of Nursing, Sultan Qaboos University, Al Khoudh, Muscat, Sultanate of Oman

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.

Publisher

SAGE Publications

Subject

Pediatrics,Pediatrics, Perinatology and Child Health

Reference27 articles.

1. Laimer M, Bauer J, Murrell D. [webpage on the Internet]. Epidemiology, Pathogenesis, Classification, and Clinical Features of Epidermolysis Bullosa. Up-to date®; 2018. Accessed August 27, 2018. https://www.uptodate.com/contents/epidemiology-pathogenesis-classification-and-clinicalfeatures-of-epidermolysis-bullosa

2. The international dystrophic epidermolysis bullosa patient registry: An online database of dystrophic epidermolysis bullosa patients and their COL7A1 mutations

3. Inherited epidermolysis bullosa: New diagnostic criteria and classification

4. Diagnosis, treatment and management of epidermolysis bullosa

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