New pathogenetic approaches to the treatment of wounds in congenital epidermolysis bullosa

Congenital epidermolysis bullosa is a group of rare, incurable, genetically heterogeneous diseases that are characterized by the fragility of the skin and mucous membranes with the formation of bullae and erosions in response to minor injury. This severe disease is divided into 4 major types and is often accompanied by multisystem complications such as anemia, gastrointestinal tract lesions, growth retardation and high risk of developing squamous cell carcinoma, which significantly deteriorates the quality of life of patients and the prognosis of disease. Gel containing a complex of triterpenes is the only pathogenetic drug for the treatment of wounds in dystrophic and junctional epidermolysis bullosa registered in Russia. The mechanism of action of the drug is aimed at the key elements of pathogenesis: modulation of inflammation, stimulation of keratinocyte migration and stabilization of dermal-epidermal junction. The international EASE study has shown that this gel significantly increases the probability of complete wound healing, shortens dressing time and reduces pain in patients. Based on these data, Russian experts have formed a consensus position in which they emphasize the clinical significance of the obtained results and predict that the drug will occupy an important place in the baseline therapy of the disease, marking the transition from a purely symptomatic approach to targeted pathogenetic exposure. The inclusion of this approach in clinical guidelines is considered as a key step to improve the effectiveness of care for patients with congenital epidermolysis bullosa in Russia.