ppharmПедиатрическая фармакологияPediatric pharmacology1727-57762500-3089Издательство «ПедиатрЪ»10.15690/pf.v19i6.2476ppharm-2230Research ArticleКЛИНИЧЕСКИЙ СЛУЧАЙCASE REPORTПруригинозная форма дистрофического буллезного эпидермолиза: клинический случайPruriginosa Pattern of Dystrophic Epidermolisys Bullosa: Clinical Casehttps://orcid.org/0000-0001-5739-0941ЛеоноваМ. А.LeonovaMaria A.

Леонова Мария Алексеевна, младший научный сотрудник лаборатории патологии кожи у детей отдела научных исследований в педиатрии

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MD

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dr.maria.leonova@gmail.comhttps://orcid.org/0000-0003-2252-8570МурашкинН. Н.MurashkinNikolay N.

Мурашкин Николай Николаевич, д.м.н., профессор

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MD, PhD, Professor

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m_nn2001@mail.ruФГАУ НМИЦ здоровья детей; РНИМУ им. Н.И. ПироговаРоссияNational Medical Research Center of Children’s Health; Pirogov Russian National Research Medical UniversityRussian FederationФГАУ НМИЦ здоровья детей; Первый МГМУ им. И.М. Сеченова (Сеченовский Университет); ЦГМА Управления делами Президента РФРоссияNational Medical Research Center of Children’s Health; Sechenov First Moscow State Medical University; Central State Medical Academy of Department of Presidential AffairsRussian Federation202212012023196479483Copyright © Леонова М.А., Мурашкин Н.Н., 20232023Леонова М.А., Мурашкин Н.Н.Leonova M.A., Murashkin N.N.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.pedpharma.ru/jour/article/view/2230

Обоснование. Пруригинозный буллезный эпидермолиз относится к редкому подтипу дистрофического буллезного эпидермолиза и характеризуется выраженным зудом, сопровождающим образование папул, бляшек и узлов, преимущественно на коже нижних конечностей, имитирующих узловатую почесуху. В настоящий момент в мире задокументировано менее 100 случаев заболевания, чем обусловлена актуальность представления данного клинического случая. Описание клинического случая. Авторами приведено описание случая пруригинозной формы доминантного дистрофического буллезного эпидермолиза у пациентки 14 лет. Заключение. Диагностика данной формы заболевания крайне сложна, и в настоящее время все лечение сводится к проведению симптоматической терапии с целью купирования зуда и профилактики образования рубцов.

Background. Epidermolisys bullosa pruriginosa is a rare pattern of dystrophic epidermolisys bullosa and characterized by severe itching that accompanies the formation of papules, plaques and nodes primarily on the lower limbs skin and imitating prurigo nodularis. Nowadays, less than 100 cases of this disease are reported in the world, thus, the presentation of this clinical case is relevant. Clinical case description. The authors describe the clinical case of pruriginous pattern of dominant dystrophic bullous epidermolysis in 14-year-old female patient. Conclusion. Diagnosis of this disease pattern is extremely difficult and currently all treatment is limited to the symptomatic therapy in order to stop itching and prevent scarring.

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