OBJECTIVE

To study medical and social aspects of congenital epidermolysis bullosa (CEB) in the Russian Federation.

MATERIAL AND METHODS

We analyzed the data of the register of patients with chronic diseases of the skin and subcutaneous tissue (register of patients with CEB) of the All-Russian public organization «Russian Society of Dermatovenereologists and Cosmetologists» and the data from primary medical documentation. Descriptive statistics was used for statistical analysis.

RESULTS

The pooled database included 452 patients with CEB from 68 constituent entities of the Russian Federation by December 31, 2019. 61% of children with CEB and 89% of those with dystrohic CEB were disabled. Mean age of establishing primary disability in children with CEB was 2.5 years, median — 1 year, mode — 0 years. 43% of adults with CEB and 66% of those with dystrophic CEB had registered disability. Twenty-five percent of children and 10% of adults with CEB and disability receive dressings at the expense of regional budgets of the constituent entities of the Russian Federation.

CONCLUSION

Congenital epidermolysis bullosa is a disabling disease with disability occuring in early ages. Provision of dressings for patients with congenital epidermolysis bullosa at the budgetary expense of the constituent entities of the Russian Federation is insufficient. The register of CEB patients of the All-Russian public organization «Russian Society of Dermatovenereologists and Cosmetologists» makes it possible to analyse and monitor of medical and social characteristics of CEB patients and their provision with medical products from public funds.