vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v18i1.1992vsp-2027Research ArticleКЛИНИЧЕСКИЕ НАБЛЮДЕНИЯCLINICAL OBSERVATIONSПриобретенный буллезный эпидермолиз у детей: серия клинических случаевEpidermolysis Bullosa Acquisita in Children: Case Serieshttps://orcid.org/0000-0003-2252-8570МурашкинН. Н.MurashkinNikolay N.

доктор медицинских наук, заведующий отделением дерматологии с группой лазерной хирургии;

профессор кафедры дерматовенерологии и косметологии ЦГМА;

профессор кафедры педиатрии и детской ревматологии Первого МГМУ им. И.М. Сеченова,

119991, Москва, Ломоносовский пр-т, д. 2, стр. 1

Moscow

m_nn2001@mail.ruhttps://orcid.org/0000-0002-0858-8780ОпрятинЛ. А.OpryatinLeonid A.

Москва

Moscow

https://orcid.org/0000-0002-6034-8231МатерикинА. И.MaterikinAlexander I.

Москва

Moscow

https://orcid.org/0000-0002-8232-8936АмбарчянЭ. Т.AmbarchyanEduard T.

Москва

Moscow

ЕпишевР. В.EpishevRoman V.

Москва

Moscow

https://orcid.org/0000-0003-1141-9352НефёдоваМ. А.NefedovaMaria A.

Москва

Moscow

Национальный медицинский исследовательский центр здоровья детейРоссияNational Medical Research Center of Children’s HealthRussian Federation2019110420191815664Copyright © Мурашкин Н.Н., Опрятин Л.А., Материкин А.И., Амбарчян Э.Т., Епишев Р.В., Нефёдова М.А., 20192019Мурашкин Н.Н., Опрятин Л.А., Материкин А.И., Амбарчян Э.Т., Епишев Р.В., Нефёдова М.А.Murashkin N.N., Opryatin L.A., Materikin A.I., Ambarchyan E.T., Epishev R.V., Nefedova 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://vsp.spr-journal.ru/jour/article/view/2027Обоснование

Обоснование. Приобретенный буллезный эпидермолиз (ПБЭ) — хроническое заболевание, сопровождаемое образованием субэпидермальных пузырей на коже и слизистых оболочках в результате аутоиммунной агрессии к коллагену VII типа. Диагностика ПБЭ у детей затруднена из-за схожести клинической картины с другими пузырными дерматозами детского возраста.

Описание клинического случая. Представлено описание трех клинических случаев ПБЭ у детей. Показано, что для постановки правильного диагноза необходимо оценить клинические данные и определить глубину залегания пузырей по данным гистологического исследования биоптата кожи. Отложение IgG относительно базальной мембраны дермы по данным реакции непрямой иммунофлюоресценции помогает установить окончательный диагноз и определить тактику ведения пациента. В лечении детей с ПБЭ применялся препарат дапсон, показавший себя эффективным и безопасным средством первого выбора при ведении таких случаев.

Заключение

Заключение. Представлен алгоритм дифференциальной диагностики ПБЭ у детей с другими пузырными заболеваниями детского возраста. Описаны результаты успешной лекарственной терапии заболевания. 

Background

Background. Epidermolysis bullosa acquisita (EBA) is chronic disease accompanied with subepidermal blistering on skin and mucous membranes as a result of autoimmune aggression to type VII collagen. EBA diagnostics in children is complicated due to similarity of clinical presentation with other bullous dermatosis in children.

Clinical Case Description. The description of three clinical cases of EBA in children is provided. It is shown that for establishing the diagnosis it is necessary to estimate clinical evidence and to define the depth of blisters according to the results of histological examination of skin biopsy sample. Determination of IgG deposition positions relatively to the skin basal membrane due to performed indirect immunofluorescence test helps us to establish final diagnosis and specify patient management. Medical drug Dapsone was used in children with EBA, it has shown to be effective and safe to use as the first-line drug in management of such patients.

Conclusion

Conclusion. The algorithm for EBA differential diagnosis with other bullous dermatosis in children is provided. Successful results of medical treatment are described. 

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