Prabhakaran Harshita, Arulappan Judie, Elizabeth J Sheeba, Cyril Vincent Sophia
Department of Maternal and Child Health, College of Nursing, Sultan Qaboos University, Al Khoudh, Muscat, Sultanate of Oman.
Glob Pediatr Health. 2023 Feb 9;10:2333794X231153507. doi: 10.1177/2333794X231153507. eCollection 2023.
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.
大疱性表皮松解症(EB)是一种与皮肤脆弱相关的极为罕见的遗传性皮肤病。它会导致皮肤上形成水疱。本文旨在更新一名营养不良性EB(DEB)患儿从婴儿期存活至学龄前,后因反复皮肤水疱、骨髓移植及生命支持措施而死亡的病程进展。进行了病例分析以评估该患儿的病程。患儿母亲签署了书面知情同意书,并允许公布患儿的详细情况及图片,同时不披露识别信息。EB的管理需要多学科团队协作。对患儿的护理应旨在保护其皮肤免受损伤、提供营养支持、精心进行伤口护理以及根据需要处理并发症。预后因病例而异。