A case of a patient with severe epidermolysis bullosa surviving to adulthood
Authors Hubail AR, Belkharoeva RK, Tepluk NP, Grabovskaya OV
Received 17 July 2018
Accepted for publication 13 September 2018
Published 15 November 2018 Volume 2018:11 Pages 413—421
Checked for plagiarism Yes
Review by Single anonymous peer review
Peer reviewer comments 3
Editor who approved publication: Dr Scott Fraser
Amal R Hubail, Roza K Belkharoeva, Natalya P Tepluk, Olga V Grabovskaya
Department of Skin and Venereal Diseases, I.M. Sechenov First Moscow State Medical University, Moscow, Russia
Purpose: The aim of this study was to evaluate the progression of a case of a patient with epidermolysis bullosa (EB) since early age who survived to adulthood, presenting with recurrent skin blisters and disfiguring scars and disabling musculoskeletal deformities.
Background: EB is a rare group of inherited diseases that affect the skin fragility causing it to blister in response to even minor trauma. Established novel treatments are limited in the literature due to its rarity, and more research is needed to set a global management approach. Clinical manifestations range widely from localized to generalized blistering.
Methods: A rare case of EB surviving to adulthood despite the complications, which has been evaluated, treated during a relapse, and followed up.
Conclusion: The described case is of considerable clinical interest due to its rarity and severity. Optimal management requires a multidisciplinary approach and revolves around the protection of the skin against slightest injury, use of careful wound care dressings, aggressive nutritional support, and early medical or surgical interventions if needed to manage any complications. Prognosis varies considerably depending on each case.
Keywords: newborn epidermolysis bullosa, defect of type VII collagen, blistering skin diseases, skin fragility
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