Prevalence of hereditary epidermolysis bullosa in the Russian Federation

2017

Vestnik Dermatologii i Venerologii, Vol 0, Iss 3, Pp 21-30 (2017)

Goal. To study the prevalence of hereditary epidermolysis bullosa (HEB) in the Russian Federation. Materials and methods. The data on the number of HEB patients by subjects of the Russian Federation as well as their age and disease type were obtained based on questionnaires. The questionnaires were sent to dermatovenerology healthcare institutions from 85 subjects of the Russian Federation. The HEB prevalence rates in the subjects of the Russian Federation were calculated as the ratio of the number of HEB patients in the subject of the Russian Federation to the population size in the subject per million residents. Results. Information about 438 HEB patients (210 female and 228 male) aged 0-66 was collected in 70 subjects of the Russian Federation as of January 1, 2015. The disease type was specified for 139 patients (86 patients with common HEB, 50 patients with dystrophic HEB and 3 patients with the borderline form of HEB). No details about the disease type were provided for 299 patients (68.26%). The HEB prevalence rate in the subjects of the Russian Federation varies from 0 to 19.73 cases per million residents. Conclusion. These data on the HEB prevalence rate in 70 subjects of the Russian Federation are comparable to the data specified in literature sources providing information on the HEB prevalence rate in some foreign states. The data represent the first step to the development of clinical recommendations concerning HEB management and establishment of a register of in the Russian Federation.

article

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Russian

State Scientific Center of Dermatovenereology and Cosmetology, 2017.

врожденный буллезный эпидермолиз, эпидемиология, распространенность·, hereditary epidermolysis bullosa, epidemiology, prevalence, Dermatology, RL1-803