Wound status in children with dystrophic epidermolysis bullosa

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Abstract

Background: Dystrophic epidermolysis bullosa (DEB) is a rare genetic disorder characterized by the formation of blisters and erosions with minimal trauma. It leads to chronic wounds and scars that limit activity and reduce quality of life. Despite the availability of international guidelines for the care of congenital epidermolysis bullosa (CEB), systematic data on chronic wound healing and temporary load-bearing in Russia are limited.

Objective: To describe the clinical characteristics of wound healing and the timeframe of care in patients with the dystrophic form of VBE.

Materials and Methods: This observational study included 46 children aged 7–18 years with confirmed VBE. Disease severity (based on the BEBS index), epithelialization time for typical and chronic (>3 months) wounds, duration of remission, relapse rate, and dressing change time were assessed. Data were obtained during hospitalization at the National Medical Research Center for Children’s Health of the Russian Ministry of Health and from the rare disease registry of the BELA. Butterfly Children Charitable Foundation. Normal distribution was tested using the Shapiro-Wilk test. Data were expressed as mean ± standard deviation (95% CI) or mean (Q1–Q3). Group comparisons were performed using the Student’s t-test, ANOVA, Mann-Whitney U-test, and Kruskal-Wallis test. Correlations were assessed using Spearman’s rank order, prognosis using linear regression, and discriminatory ability using receiver operating characteristic (ROC) analysis using the Youden index.

Results: Average age was 12.1±3.2 years, BEBS was 24.4±11.5. Chronic wounds were observed in 34.8% of patients, and recurrent lesions were observed in 97.8%. The average wound care time was 2.0±1.3 hours/day and increased with severity (ρ=0.891; p<0.001). ROC analysis (AUC=0.805; p<0.001) confirmed the predictive value of BEBS for the risk of chronic wound development.

Conclusion: DEB is accompanied by chronic and recurrent wounds requiring constant wound care. As the disease severity increases, both the duration of wound healing and the amount of daily workload increase, emphasizing the need for multidisciplinary support for patients and their families.

About the authors

Bova V. Erentsenova

National Scientific and Practical Center for Children’s Health

Author for correspondence.
Email: berentsenova@deti-bela.ru
ORCID iD: 0009-0007-7247-6859

Postgraduate Student

Russian Federation, Moscow

Olga S. Orlova

National Scientific and Practical Center for Children’s Health; Charitable Foundation “BELA. Butterfly Children”

Email: berentsenova@deti-bela.ru
ORCID iD: 0009-0002-6642-5776

Cand. Sci. (Med.), Dermatovenerologist; Consultant

Russian Federation, Moscow; Moscow

Roman V. Epishev

National Scientific and Practical Center for Children’s Health; Russian Medical Academy of Continuous Professional Education

Email: berentsenova@deti-bela.ru
ORCID iD: 0000-0002-4107-4642

Cand. Sci. (Med.), Dermatovenerologist; Assistant Professor, Department of Dermatovenerology and Cosmetology

Russian Federation, Moscow; Moscow

Vladislav V. Chernikov

National Scientific and Practical Center for Children’s Health

Email: berentsenova@deti-bela.ru
ORCID iD: 0000-0002-8750-9285

Cand. Sci. (Med.), Head of the Methodological Accreditation and Simulation Center, Head of the Department, Pediatrician, Department of Diagnostics and Rehabilitation Treatment, Associate Professor, Department of Pediatrics and Public Health

Russian Federation, Moscow

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