Follow-up and treatment of comorbid conditions in patients with epidermolysis bullosa

Follow-up and treatment of comorbid conditions in patients with epidermolysis bullosa

K.M. Gadzhimuradova¹, I.E. Torshina², M.N. Gadzhimuradov¹, M.G. Magomedov¹, G.D. Mamasheva¹

¹Dagestan State Medical University, Makhachkala

²Saratov State Medical University, Smolensk

Background: inherited epidermolysis bullosa (IEB) is a heterogeneous multisystem genodermatosis. A multidisciplinary team plays a great role in its clinical follow-up. At the same time, there are significant differences in regional populations. This determines a specific approach to the diagnosis and treatment of each regional patient population with IEB.

Aim: to study comorbidity in patients with IEB and methods for the patient follow-up in the Republic of Dagestan.

Patients and Methods: methods for the follow-up of the entire patient population with IEB (n=131) were studied. The analysis of comorbid pathology was conducted in 18 patients suffering from IEB, as well as a comparative study of patient follow-up results with IEB, depending on the supervising specialist, was performed. We studied the change in the Birmingham Epidermolysis Bullosa Severity score (BEBS) in the setting of the follow-up in the study group (n=18). The environmental situation impact on the disease course was also assessed.

Results: preventive monitoring and treatment of multiple organ pathology is not conducted in patients with IEB in the Republic of Dagestan. Mobile patients with mild to moderate severity mainly applied to primary care facilities, receiving treatment from a neurologist, pulmonologist, traumatologist and orthopedist. A significant part of the patients did not receive counseling from dedicated specialists. Patients with severe lesions traveled outside the republic to federal medical centers or sought help from charitable funds. The provision of medical care with the participation of dedicated specialists in 15/17 (88,24±7,81%) patients led to a significant decrease in BEBS.

Conclusion: the involvement of experts in the patient management with IEB stabilized the skin process and significantly reduced BEBS. It is recommended to involve specialists using remote telemedicine technologies for the follow-up of patients with IEB. In recessive dystrophic epidermolysis bullosa, the result of symptomatic therapy significantly depended on the environmental situation in the patient’s place of residence.

Keywords: ecology, comorbidity, inherited epidermolysis bullosa, genodermatosis, telemedicine technologies, birch-bark triterpenoids, betulin.

For citation: Gadzhimuradova K.M., Torshina I.E., Gadzhimuradov M.N., Magomedov M.G., Mamasheva G.D. Follow-up and treatment of comorbid conditions in patients with epidermolysis bullosa. RMJ. 2024;7:3–11.