Wound status in children with dystrophic epidermolysis bullosa
Erentsenova B.V., Orlova O.S., Epishev R.V., Chernikov V.V.
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.
For citations: Erentsenova B.V., Orlova O.S., Epishev R.V., Chernikov V.V. Wound status in children with dystrophic epidermolysis bullosa. Pharmateca. 2025;32(10):123-128. (In Russ.). DOI: https://dx.doi.org/10.18565/pharmateca.2025.10.123-128
Authors’ contribution: Erentsenova B.V. – participation in the development of the concept and purpose of the study, statistical analysis of data, interpretation of results, writing the article, and scientific editing of the manuscript. Orlova O.S. – participation in the development of the concept and purpose of the study, development of the study design and methodology, scientific editing and critical review of the manuscript. Epishev R.V. – participation in the development of the concept and purpose of the study, collection of clinical and follow-up data. Chernikov V.V. – participation in the development of the concept and purpose of the study, statistical analysis of data, and interpretation of results. All authors approved the final version of the article and are responsible for all aspects of the work.
Conflicts of interest: The authors confirm that they have no conflicts of interest to declare.
Funding: The study was conducted without any sponsorship.
Ethical Approval: The study protocol was approved by the Ethics Committee of the National Medical Research Center for Children’s Health (Protocol No. 7 dated July 17, 2025).
Patient Consent for Publication: All patients provided informed consent for the publication of their data.
Authors’ Data Sharing Statement: The data supporting the findings of this study are available upon request from the corresponding author after approval from the principal investigator.
Keywords
congenital epidermolysis bullosa
chronic wounds
healing
wound care
quality of life
medical and social care
About the Authors
Bova V. Erentsenova, Postgraduate Student, National Scientific and Practical Center for Children’s Health, Moscow, Russia;berentsenova@deti-bela.ru, ORCID: https://orcid.org/0009-0007-7247-6859 (corresponding author)
Olga S. Orlova, Cand. Sci. (Med.), Dermatovenerologist, National Scientific and Practical Center for Children’s Health; Consultant, Charitable Foundation “BELA. Butterfly Children”, Moscow, Russia; ORCID: https://orcid.org/0009-0002-6642-5776
Roman V. Epishev, Cand. Sci. (Med.), Dermatovenerologist, National Scientific and Practical Center for Children’s Health; Assistant Professor, Department of Dermatovenerology and Cosmetology, Russian Medical Academy of Continuous Professional Education, Moscow, Russia; ORCID: https://orcid.org/0000-0002-4107-4642
Vladislav V. Chernikov, 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, National Scientific and Practical Center for Children’s Health, Moscow, Russia; ORCID: https://orcid.org/0000-0002-8750-9285
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