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Int J Gen Med. 2018 Nov 15;11:413-421. doi: 10.2147/IJGM.S180464. eCollection 2018.

A case of a patient with severe epidermolysis bullosa surviving to adulthood.

Author information

1
Department of Skin and Venereal Diseases, I.M. Sechenov First Moscow State Medical University, Moscow, Russia, dr.amalhubail@gmail.com.

Abstract

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:

blistering skin diseases; defect of type VII collagen; newborn epidermolysis bullosa; skin fragility

Conflict of interest statement

Disclosure The authors report no conflicts of interest in this work.

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