According to the data of the Federal State Budgetary Institution «State Research Center for Diseases» of the Ministry of Health of the Russian Federation in the Republic of Dagestan, the prevalence of CEB is 19.73 per 1.000.000. As a result of the revision of the CEB patients registered at the medical facility, this indicator was 48.7 per 1.000.000 populations. Every second patient with CEB in the region dropped out of the statistical record. At the same time, 103 CEB patients are listed in the register of the republican center of genetics. There is no interaction between specialists of related specialties in the provision of medical care to patients with CEB. Only 36% (24.7±3.57) were provided with medical devices and 34% (23.3±3.5) patients with CEB were not observed at the level of the outpatient clinic.
Objective is to study the clinical features of CEB, to organize effective dispensary observation and supply of medical devices (SMD) for patients with this genodermatosis in large cities of the Republic of Dagestan.
MATERIALS AND METHODS
We examined 34 patients with a diagnosis of congenital epidermolysis bullosa living in the cities of Makhachkala, Kaspiysk and Derbent, and trained their caregivers in the methods of skin treatment.
Results and conclusions. Complications of the skin, organs and systems, which can be considered as pathognomonic signs of CEB and used in the verification of this disease, were identified. The patients were divided into 4 groups according to the severity of CEB and correspondingly they were recommended an indicative set of medical devices. It was found that in the first year of life, 33% (97.1±0.84) of children have clinical manifestations of CEB, and in the second year, 91.18±4.86%, and in the subsequent years of life, 67.65±8.02% clinical symptoms are increasing. This allows in dynamic observation to clarify the type and subtype of CEB, to organize timely the prevention of skin complications and associated diseases. Baths with herbs (chamomile, string, calendula, oak bark) and local cooling lotions contribute to the stabilization and regression of the pathological process of the skin.
