medalphabetМедицинский алфавитMedical alphabet2078-56312949-2807ООО «Альфмед»10.33667/2078-5631-2022-8-89-91medalphabet-2612Research ArticleДЕТСКАЯ ДЕРМАТОЛОГИЯPEDIATRIC DERMATOLOGYСовременные представления об этиологических факторах развития задержки полового развития у детей с врожденным буллезным эпидермолизомCurrent representations about etiological factors in development of delayed puberty in children with inherited epidermolysis bullosahttps://orcid.org/0000-0001-5739-0941ЛеоноваМ. А.LeonovaM. A.

 Леонова Мария Алексеевна, м.н.с. лаборатории патологии кожи у детей НИИ детской дерматологии

 Leonova Maria A., junior researcher at Laboratory of Skin Pathology in Children Research Institute of Pediatric Dermatology 

dr.maria.leonova@gmail.comФГАУ «Национальный медицинский исследовательский центр здоровья детей» Минздрава России; ФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Минздрава РоссииРоссияNational Medical Research Centre for Children’s Health; Russian National Research Medical University n.a. N.I. PirogovRussian Federation20221805202208Дерматология (1)8991Copyright © Леонова М.А., 20222022Леонова М.А.Leonova M.A.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.med-alphabet.com/jour/article/view/2612

Врожденный буллезный эпидермолиз характеризуется широким спектром клинических проявлений: при легком течении от единичных пузырей и эрозий, преимущественно с акральной локализацией, незначительно влияющих на качество и продолжительность жизни пациентов, до тяжелых инвалидизирующих форм с высоким уровнем смертности от недостаточности питания, септицемии в раннем возрасте и высокого риска развития плоскоклеточной карциномы кожи у взрослых пациентов. По данным многочисленных исследований, у детей, страдающих хроническими заболеваниями, часто отмечается задержка полового созревания. На сегодняшний день нет достоверных данных о частоте встречаемости задержки полового созревания у детей с врожденным буллезным эпидермолизом, что представляет интерес для дальнейших исследований с целью более индивидуализированного подхода к лечению и улучшения качества жизни пациентов.

Inherited epidermolysis bullosa is distinguished by a wide range of clinical manifestations: from single blisters and erosions, mainly with acral localization, which insignifcantly affect the quality and life expectancy of patients with a mild course of the disease, to severe disabling forms with a high mortality rate from multifactorial malnutrition, septicemia in early age and high risk of developing squamous cell carcinoma of the skin in adult patients. According to research studies, in children suffering from chronic diseases, there is often a delay of puberty. To date, there are no reliable data on the incidence of delayed puberty in children with inherited epidermolysis bullosa, which is of interest for further research in order to more individualized approach to treatment and improve the quality of life of patients.

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The authors declare that there are no conflicts of interest present.