medsovetМедицинский СоветMeditsinskiy sovet = Medical Council2079-701X2658-5790REMEDIUM GROUP Ltd.10.21518/ms2025-265medsovet-9372Research ArticleДЕРМАТОЛОГИЯDERMATOLOGYОсобенности терапии кожного мастоцитоза у детейFeatures of the treatment of cutaneous mastocytosis in childrenhttps://orcid.org/0000-0002-0767-8821КасихинаЕ. Н.KasikhinaE. I.

Касихина Елена Игоревна, к.м.н., доцент, доцент кафедры дерматовенерологии, аллергологии и косметологии Медицинского института, Российский университет дружбы народов имени Патриса Лумумбы; врач-дерматовенеролог, Московский научно-практический центр дерматовенерологии и косметологии

117198, Москва, ул. Миклухо-Маклая, д. 6; 119071, Москва, Ленинский проспект, д. 17

Elena I. Kasikhina, Cand. Sci. (Med.), Associate Professor, Associate Professor of the Department of Dermatovenereology, Allergology and Cosmetology, Medical Institute, Peoples’ Friendship University of Russia named after Patrice Lumumba; Dermatologist, Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology

6, Miklukho-Maklai St., Moscow, 117198; 17, Leninsky Ave., Moscow, 119071

kasprof@bk.ruhttps://orcid.org/0000-0003-3386-1467ОстрецоваМ. Н.OstretsovaM. N.

Острецова Мария Николаевна, к.м.н., доцент, доцент кафедры дерматовенерологии, аллергологии и косметологии Медицинского института 

117198, Москва, ул. Миклухо-Маклая, д. 6

Maria N. Ostretsova, Cand. Sci. (Med.), Associate Professor, Associate Professor of the Department of Dermatovenereology, Allergology and Cosmetology, Medical Institute

6, Miklukho-Maklai St., Moscow, 117198

ostretsova-mn@rudn.ruhttps://orcid.org/0000-0002-9578-5490ПотекаевН. Н.PotekaevN. N.

Потекаев Николай Николаевич, д.м.н., директор, Московский научно-практический центр дерматовенерологии и косметологии;  профессор, заведующий кафедрой кожных болезней и косметологии Института непрерывного образования и профессионального развития, Российский национальный исследовательский медицинский университет имени Н.И. Пирогова

119071, Москва, Ленинский проспект, д. 17; 117997, Москва, ул. Островитянова, д. 1

Nikolay N. Potekaev, Dr. Sci. (Med.), Director, Moscow Scientific and Practical Center of Dermatovenereology and Cosmetology; Professor, Head of the Department of Skin Diseases and Cosmetology, Institute of Continuing Education and Professional Development, Pirogov Russian National Research Medical University

17, Leninsky Ave., Moscow, 119071; 1, Ostrovityanov St., Moscow, 117997

klinderma@mail.ruМосковский научно-практический центр дерматовенерологии и косметологии; Российский университет дружбы народов имени Патриса ЛумумбыРоссияMoscow Scientific and Practical Center of Dermatovenereology and Cosmetology; Peoples’ Friendship University of Russia named after Patrice LumumbaRussian FederationРоссийский университет дружбы народов имени Патриса ЛумумбыРоссияPeoples’ Friendship University of Russia named after Patrice LumumbaRussian FederationМосковский научно-практический центр дерматовенерологии и косметологии; Российский национальный исследовательский медицинский университет имени Н.И. ПироговаРоссияMoscow Scientific and Practical Center of Dermatovenereology and Cosmetology; Pirogov Russian National Research Medical UniversityRussian Federation2025100920251913250256Copyright © Касихина Е.Н., Острецова М.Н., Потекаев Н.Н., 20252025Касихина Е.Н., Острецова М.Н., Потекаев Н.Н.Kasikhina E.I., Ostretsova M.N., Potekaev N.N.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-sovet.pro/jour/article/view/9372

Мастоцитоз – гетерогенная группа заболеваний, характеризующихся накоплением клональных тучных клеток в тканях. Мастоцитозу присущи сложный патогенез, неоднородная клиническая картина и неоднозначный прогноз. Сложные молекулярные механизмы, мультиорганное поражение и вариабельное клиническое течение заболевания требуют междисциплинарных подходов к диагностике и разработке стратегии его лечения. У пациентов с симптомами, связанными с медиаторами тучных клеток, большое значение имеет строгое избегание любых триггеров, приводящих к активации и дегрануляции тучных клеток. Лечение кожного мастоцитоза у детей не имеет стандартных рекомендаций и должно учитывать клиническую форму и тип заболевания, тяжесть медиаторных симптомов, возраст, сопутствующие и коморбидные заболевания. Этиотропная лекарственная терапия кожного мастоцитоза пока не разработана, поэтому в педиатрической практике используются средства для симптоматического лечения заболевания. В статье систематизированы данные международного опыта ведения детей с кожными и системными медиаторными симптомами различной тяжести. Дана характеристика (механизм действия, показания) групп антимедиаторных препаратов, наиболее часто применяющихся в амбулаторной педиатрической практике. Сделан акцент на необходимость проведения коррекции уровня витамина D у детей с мастоцитозом. Проанализированы данные, посвященные периоперационному ведению детей с кожным мастоцитозом. Уходовая и наружная терапия является актуальной для клинических форм заболевания, сопровождающихся формированием волдырей, пузырей и зудом. Наблюдение детей должно осуществляться специалистами-дерматовенерологами и аллергологамииммунологами, имеющими опыт работы с данной патологией. Детям с кожным мастоцитозом может потребоваться психологическая или психиатрическая помощь. Семьи пациентов должны быть информированы об особенностях клинической картины, необходимости динамического наблюдения, актуальности проведения профилактической и симптоматической терапии и прогнозе заболевания. Своевременно назначенная антимедиаторная терапия позволит предупредить развитие тяжелых медиаторных симптомов, повысить качество жизни и улучшить прогноз у детей с кожным мастоцитозом.

Mastocytosis is a heterogeneous group of diseases with accumulation of clonal mast cells in tissues. Mastocytosis is characterized by complex pathogenesis, diverse clinical picture and ambiguous prognosis. Complex molecular mechanisms, multiorgan damage and variable clinical course require interdisciplinary approaches to diagnostics and development of treatment strategy for mastocytosis. In patients with symptoms associated with mast cell mediators, strict avoidance of any triggers leading to activation and degranulation of mast cells is of great importance. Treatment of cutaneous mastocytosis in children does not have standard recommendations and should consider the clinical form and type of cutaneous mastocytosis, severity of mediator symptoms, age, concomitant and comorbid diseases. Etiotropic drug therapy for cutaneous mastocytosis has not yet been developed, therefore, in pediatric practice, agents for symptomatic treatment of the disease are used. The article systematizes the data of international experience in the management of children with cutaneous and systemic mediator symptoms of varying severity. The characteristics (mechanism of action, indications) of groups of antimediator drugs most often used in outpatient pediatric practice are given. Emphasis is placed on the need to correct the level of vitamin D in children with mastocytosis. Data on the perioperative management of children with cutaneous mastocytosis are analyzed. Skin care and external therapy are relevant for clinical forms of the disease, accompanied by the formation of weals, bullae and itching. Observation of children should be carried out by specialists-dermatovenerologists and allergists-immunologists, with experience in working with this pathology. Children with cutaneous mastocytosis may require psychological or psychiatric help. Families of patients should be informed about the clinical features, the need for dynamic observation, the relevance of preventive and symptomatic therapy, and the prognosis of the disease. Timely prescribed anti-mediator therapy will prevent the development of severe mediator symptoms, improve the quality of life, and improve the prognosis in children with cutaneous mastocytosis.

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