Vestnik dermatologii i venerologiiVestnik dermatologii i venerologiiВестник дерматологии и венерологии0042-46092313-6294Rossijskoe Obschestvo Dermatovenerologov i Kosmetologov1679410.25208/vdv16794REVIEWSОБЗОР ЛИТЕРАТУРЫReview ArticleApplication of biomedical cell products in the treatment of congenital epidermolysis bullosaПрименение эквивалентов кожи в терапии врожденного буллезного эпидермолизаhttps://orcid.org/0000-0003-3805-84893604-6491KaramovaArfenya E.КарамоваАрфеня Эдуардовна
Russian Federation

MD, Cand. Sci. (Med.), Assistant Professor

к.м.н., доцент

karamova@cnikvi.ruhttps://orcid.org/0000-0002-2924-30368310-7019AulovaKseniya M.АуловаКсения Максимовна
Russian Federation
aulovaksenia@mail.ruhttps://orcid.org/0000-0002-9688-27273385-4723ChikinVadim V.ЧикинВадим Викторович
Russian Federation

MD, Dr. Sci. (Med.)

д.м.н.

chikin@cnikvi.ruhttps://orcid.org/0000-0002-7625-05038771-4990KubanovАlexey A.КубановАлексей Алексеевич
Russian Federation

MD, Dr. Sci. (Med.), Professor, Academician of the Russian Academy of Sciences

д.м.н., профессор, академик РАН

alex@cnikvi.ruState Research Center of Dermatovenereology and CosmetologyГосударственный научный центр дерматовенерологии и косметологии2510202422112024100521310406202413092024Copyright ©; 2024, Karamova A.E., Aulova K.M., Chikin V.V., Kubanov А.A.Copyright ©; 2024, Карамова А.Э., Аулова К.М., Чикин В.В., Кубанов А.А.2024Karamova A.E., Aulova K.M., Chikin V.V., Kubanov А.A.Карамова А.Э., Аулова К.М., Чикин В.В., Кубанов А.А.https://creativecommons.org/licenses/by-nc/4.0https://vestnikdv.ru/jour/article/view/16794

Congenital epidermolysis bullosa is a phenotypically and genetically heterogeneous group of genodermatoses, which are characterized by decreasing of skin’s structural protein production up to complete absence or violation of the structure as a result of mutation. Congenital epidermolysis bullosa clinically manifests by the development of blisters on the skin and mucous membranes after mechanical injury. The presence of long-term erosive and ulcerative defects in patients with congenital epidermolysis bullosa reduces the quality of patients’ life and also leads to malignancy of the lesions, as a result of constant stimulation of regenerative processes. Currently, in the treatment of patients with congenital epidermolysis bullosa symptomatic therapy, including prevention of secondary infections, use of pain medication and atraumatic non-adherent wound dressings and correction of complications, is widely used. Nevertheless there are a lot of publications describing the use of skin substitutes, three-dimensional tissue-engineered structures based on auto- and allogeneic cells that promote rapid epithelization of wounds in patients with congenital epidermolysis bullosa. Tissue-engineered skin substitutes can be categorised based on their cellular composition: epidermal substitutes consist of an epidermal layer of stratified keratinocyte sheets with or without an underlying acellular dermal layer containing scaffolding; dermal substitutes contain fibroblasts embedded within a scaffolded dermal matrix; composite substitutes are composed of an epidermal layer of stratified keratinocyte sheets and an underlying scaffolded dermal layer containing fibroblasts. The most prospective and at the same time the least studied direction of congenital epidermolysis bullosa treatment is the use of a combined skin equivalent created on the basis of keratinocytes and fibroblasts, which mixes the advantages of epidermal and dermal grafts.

Врожденный буллезный эпидермолиз — фенотипически и генетически гетерогенная группа генодерматозов, при которых в результате мутаций уменьшается продукция структурных белков кожи вплоть до полного отсутствия либо нарушается их структура. Основным клиническим проявлением врожденного буллезного эпидермолиза являются пузыри, возникающие на коже и слизистых оболочках после незначительного механического воздействия. Наличие длительно существующих эрозивно-язвенных дефектов у пациентов с врожденным буллезным эпидермолизом не только снижает качество жизни больных, но и в результате постоянной стимуляции регенеративных процессов приводит к малигнизации очагов. В настоящее время для лечения пациентов с врожденным буллезным эпидермолизом применяется симптоматическая терапия, включающая в себя профилактику вторичного инфицирования эрозивно-язвенных дефектов, применение обезболивающих препаратов, использование атравматичных неадгезивных перевязочных средств и коррекцию осложнений. На сегодняшний день опубликовано большое количество работ, описывающих применение эквивалентов кожи, трехмерных тканеинженерных конструкций на основе ауто- и аллогенных клеток, способствующих быстрой эпителизации раневых поверхностей у больных врожденным буллезным эпидермолизом. Эквиваленты кожи подразделяются на эпидермальные, содержащие кератиноциты с подлежащей бесклеточной дермальной матрицей либо без нее, дермальные, созданные на основе фибробластов, встроенных в дермальную матрицу, и комбинированные, сочетающие в себе кератиноциты и дермальную матрицу с фибробластами. Наиболее перспективным и одновременно наименее изученным направлением терапии врожденного буллезного эпидермолиза является применение комбинированного эквивалента кожи, созданного на основе кератиноцитов и фибробластов, в котором сочетаются преимущества эпидермальных и дермальных трансплантатов.

epidermolysis bullosaskin substitutesskin graftingврожденный буллезный эпидермолизэквиваленты кожитканеинженерные конструкты кожиThe article is prepared and published as a part of the state task of State Research Center of Dermatovenereology and Cosmetology № 056-00003-24-02 for 2024 and the 2025–2026 planning periods “The development of a somatic cell based medicinal product for the treatment of patients with epidermolysis bullosa”.Рукопись подготовлена и опубликована в рамках выполнения государственного задания ФГБУ «ГНЦДК» Минздрава России № 056-00003-24-02 на 2024 г. и на плановый период 2025–2026 гг. «Разработка лекарственного препарата на основе соматических клеток для лечения больных врожденным буллезным эпидермолизом».Natsuga K, Shinkuma S, Hsu CK, Fujita Y, Ishiko A, Tamai K, et al. Current topics in Epidermolysis bullosa: Pathophysiology and therapeutic challenges. 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