Epidermolysis Bullosa Acquisita in Children: Case Series
Nikolay N. Murashkin,
Leonid A. Opryatin,
Alexander I. Materikin,
Eduard T. Ambarchyan,
Roman V. Epishev,
Maria A. Nefedova https://doi.org/10.15690/vsp.v18i1.1992
Abstract
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. The algorithm for EBA differential diagnosis with other bullous dermatosis in children is provided. Successful results of medical treatment are described.
About the Authors
Nikolay N. Murashkin
National Medical Research Center of Children’s Health
Russian Federation
Moscow
Disclosure of interest: Получение исследовательских грантов от фармацевтических компаний Jansen, Eli Lilly, Novartis. Получение гонораров за научное консультирование от компаний Galderna, Pierre Fabre, Bayer, Leofarma, Pfizer, AbbVie, ООО «Зелдис-Фарма».
Russian Federation
Moscow
Disclosure of interest: Получение исследовательских грантов от фармацевтических компаний Jansen, Eli Lilly, Novartis. Получение гонораров за научное консультирование от компаний Galderna, Pierre Fabre, Bayer, Leofarma, Pfizer, AbbVie, ООО «Зелдис-Фарма».
Leonid A. Opryatin
National Medical Research Center of Children’s Health
Russian Federation
Moscow
Russian Federation
Moscow
Alexander I. Materikin
National Medical Research Center of Children’s Health
Russian Federation
Moscow
Disclosure of interest: Получение гонораров за научное консультирование от компаний Eli Lilly, Novartis.
Russian Federation
Moscow
Disclosure of interest: Получение гонораров за научное консультирование от компаний Eli Lilly, Novartis.
Eduard T. Ambarchyan
National Medical Research Center of Children’s Health
Russian Federation
Moscow
Disclosure of interest: Получение гонораров за научное консультирование от компаний Eli Lilly, Novartis, Jansen.
Russian Federation
Moscow
Disclosure of interest: Получение гонораров за научное консультирование от компаний Eli Lilly, Novartis, Jansen.
Roman V. Epishev
National Medical Research Center of Children’s Health
Russian Federation
Moscow
Disclosure of interest: Получение гонораров за научное консультирование от компаний Eli Lilly, Novartis.
Russian Federation
Moscow
Disclosure of interest: Получение гонораров за научное консультирование от компаний Eli Lilly, Novartis.
Maria A. Nefedova
National Medical Research Center of Children’s Health
Russian Federation
Moscow
Russian Federation
Moscow
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Review
For citations:
Murashkin N.N., Opryatin L.A., Materikin A.I., Ambarchyan E.T., Epishev R.V., Nefedova M.A. Epidermolysis Bullosa Acquisita in Children: Case Series. Current Pediatrics. 2019;18(1):56-64. (In Russ.) https://doi.org/10.15690/vsp.v18i1.1992
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