Epidemiology and Providing of Healthcare for Patients with Inherited Epidermolysis Bullosa in the Russian Federation

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Abstract


Background: Inherited epidermolysis bullosa is a group of genetic skin disorders. In most severe forms, such as junctional and dystrophic subtypes, quality of life and life expectancy are significantly decreased. Therapeutic approaches include wound care and complication treatment.

Aims: To evaluate the incidence and prevalence of inherited epidermolysis bullosa in the Russian Federation, sociodemographic characterisrics and provision of healthcare.

Methods: To conduct the research forms summarizing demographic, medical and social information on inherited epidermolysis bullosa patients were developed. The forms were sent to state outpatient dermatologic clinics in federal subject of the Russian Federation. Data on inherited epidermolysis bullosa patients from outpatient dermatologic clinics were obtained within the period of 2014−2016 by extracting information from their medical charts. A confirmed inherited epidermolysis bullosa diagnosis was considered as an inclusion criterion for the research. Based on the collected data prevalence and incidence rate were estimated.

Results: Data on 439 patients from 70 federal subject at year-end 2014, 404 patients from 59 federal subject at year-end 2015 and 417 patients from 60 federal subject at year-end 2016 were collected. In 2014 EB simplex was diagnosed in 19.6% patients, dystrophic EB — in 11.6% patients. In most patients (66%) EB type was not diagnosed. In 2016 patients with EB simplex (48%) and dystrophic EB (24.2%) prevailed. In 25% patients an EB type was not specified. In 2014 the prevalence rates were estimated as 3.6 (in 70 federal subject), in 2015 — 3.8 (in 59 federal subject), in 2016 — 3.9 per 1 million population (in 60 federal subject). The incidence rates were estimated as 0.22 and 0.33 per 1 million population in 2015 and 2016 respectively.

Conclusions: In 2016 the percent of patients with established EB type has increased in comparison to 2014. No significant changes in prevalence rates has been registered.


Alexey A. Kubanov

State Research Center of Dermatovenereology and Cosmetology, Ministry of Healthcare of the Russian Federation

Email: alex@cnikvi.ru
ORCID iD: 0000-0002-7625-0503

Russian Federation

MD, PhD, Professor

3 bld 6, Korolenko street, 107076 Moscow

SPIN-ID: 3604-6491

 

Arfenya A. Karamova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Healthcare of the Russian Federation

Author for correspondence.
Email: karamova@cnikvi.ru
ORCID iD: 0000-0003-3805-8489

Russian Federation

MD, PhD.

Moscow

SPIN-ID: 3604-6491

 

Vadim V. Chikin

State Research Center of Dermatovenereology and Cosmetology, Ministry of Healthcare of the Russian Federation

Email: chikin@cnikvi.ru
ORCID iD: 0000-0002-9688-2727

Russian Federation

MD, PhD.

Moscow

SPIN-ID: 3385-4723

 

Elena V. Bogdanova

State Research Center of Dermatovenereology and Cosmetology, Ministry of Healthcare of the Russian Federation

Email: bogdanova@cnikvi.ru
ORCID iD: 0000-0002-0662-2682

Russian Federation

MD, PhD.

Moscow

SPIN-ID: 6372-2237

 

Ekaterina S. Monchakovskaya

State Research Center of Dermatovenereology and Cosmetology, Ministry of Healthcare of the Russian Federation

Email: monchakovskaya@cnikvi.ru
ORCID iD: 0000-0002-6402-0962

Russian Federation

MD.

Moscow

SPIN-ID: 9859-1912

 

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