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Optimal Management Of Chronic Wounds In Paediatric Junctional Epidermolysis Bullosa Patients

Authors McDonald CR, Plevey K, Petrof G, Martinez AE

Received 18 April 2019

Accepted for publication 5 September 2019

Published 3 October 2019 Volume 2019:6 Pages 99—107

DOI https://doi.org/10.2147/CWCMR.S172193

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 3

Editor who approved publication: Professor Marco Romanelli


CR McDonald, K Plevey, G Petrof, AE Martinez

Paediatric Dermatology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK

Correspondence: AE Martinez
Paediatric Dermatology, Great Ormond Street Hospital for Children NHS Foundation Trust London, WC1N 3JH, UK
Tel +207 829 780
Email Anna.martinez@gosh.nhs.uk

Abstract: Epidermolysis bullosa (EB) is a group of rare genetic mucocutaneous fragility disorders. Patients with EB can experience blister formation following minor skin trauma, which can progress to chronic wounds. These wounds can be painful and difficult to manage. There are four major types of EB which are defined by the defective protein and the layer within the skin this protein is located. Junctional epidermolysis bullosa (JEB) is caused by mutations within the lamina lucida proteins which are located at the dermoepidermal junction. The prognosis of JEB varies considerably between the different subtypes of JEB, with JEB generalised severe being the most severe, with high mortality within the first 2 years of life. On the opposite end, patients with JEB localised would be expected to have normal life expectancy. In this review, we discuss the management of chronic wounds in paediatric patients with JEB, including suitable wound cleansing, recommended dressings and topical preparations for chronic wound treatments. We include chronic wounds over specific anatomical sites which are frequent in patients with JEB and their recommended management. Important differences between the subtypes of JEB, and specifics on dressings suitable in the neonatal and infancy period are highlighted. Symptom care of wounds in these children, such as pain, infection, pruritus and overgranulation are also addressed. Finally, we highlight the changes to practice which have occurred in recent years and novel treatments which have been trialled and can be considered.

Keywords: junctional epidermolysis bullosa, paediatric JEB, wound management


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