vspВопросы современной педиатрииCurrent Pediatrics1682-55271682-5535Издательство «ПедиатрЪ»10.15690/vsp.v23i5.2794vsp-3613Research ArticleКЛИНИЧЕСКОЕ НАБЛЮДЕНИЕCLINICAL OBSERVATIONSТерапевтические подходы при диффузном кожном мастоцитозе у детей: обзор литературы и собственный клинический опытTreatment Approaches for Diffuse Cutaneous Mastocytosis in Children: Literature Review and Actual Clinical Experiencehttps://orcid.org/0000-0002-1469-2365ВалиевТ. Т.ValievTimur T.

Валиев Тимур Теймуразович, доктор медицинских наук, заведующий отделением детской онкологии и гематологии (химиотерапия гемобластозов)

115522, Москва, Каширское шоссе, 23, тел.: +7 (499) 444-24-24

Moscow

timurvaliev@mail.ruhttps://orcid.org/0000-0003-2252-8570МурашкинН. Н.MurashkinNikolay N.

Москва

Moscow

https://orcid.org/0000-0001-5911-553XБелышеваТ. С.BelyshevaTatiana S.

Москва

Moscow

https://orcid.org/0009-0009-4243-3887МисиковаЗ. Г.MisikovaZarina G.

Москва

Moscow

https://orcid.org/0000-0002-6548-569XПронинаД. А.ProninaDaria A.

Москва

Moscow

https://orcid.org/0000-0002-4107-4642ЕпишевР. В.EpishevRoman V.

Москва

Moscow

https://orcid.org/0000-0002-0081-0981ИвановР. А.IvanovRoman A.

Москва

Moscow

НМИЦ онкологии им. Н.Н. Блохина; Первый МГМУ им. И.М. Сеченова (Сеченовский Университет); РМАНПОРоссияNational Medical Research Center of Oncology named after N.N. Blokhin; Sechenov First Moscow State Medical University; Russian Medical Academy of Continuing Professional EducationRussian FederationПервый МГМУ им. И.М. Сеченова (Сеченовский Университет); НМИЦ здоровья детей; ЦГМАРоссияSechenov First Moscow State Medical University; National Medical Research Center of Children’s Health; Central State Medical Academy of Department of Presidential AffairsRussian FederationНМИЦ онкологии им. Н.Н. БлохинаРоссияNational Medical Research Center of Oncology named after N.N. BlokhinRussian FederationРМАНПОРоссияRussian Medical Academy of Continuing Professional EducationRussian FederationЦГМАРоссияCentral State Medical Academy of Department of Presidential AffairsRussian FederationНМИЦ здоровья детейРоссияNational Medical Research Center of Children’s HealthRussian Federation202401112024235384390Copyright © Валиев Т.Т., Мурашкин Н.Н., Белышева Т.С., Мисикова З.Г., Пронина Д.А., Епишев Р.В., Иванов Р.А., 20242024Валиев Т.Т., Мурашкин Н.Н., Белышева Т.С., Мисикова З.Г., Пронина Д.А., Епишев Р.В., Иванов Р.А.Valiev T.T., Murashkin N.N., Belysheva T.S., Misikova Z.G., Pronina D.A., Epishev R.V., Ivanov R.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/3613

Обоснование. Мастоцитоз является весьма редким заболеванием с большим клиническим разнообразием проявлений, в основе которых лежит аномальная клональная пролиферация тучных клеток в органах и тканях, включая кожу, костный мозг, лимфатические узлы, печень, селезенку и желудочно-кишечный тракт. Диагноз устанавливается на основании клинических проявлений, лабораторных и инструментальных методов исследования. Особую роль в постановке диагноза «мастоцитоз» играют симптом Дарье – Унны и гистологическое исследование. Представленный клинический случай описывает весьма редкую кожную форму мастоцитоза. Описание клинического случая. Девочка, возраст 2,5 года, госпитализирована с множественными эритематозными папулами на теле, лице и конечностях. Комплексное обследование, включая трепанобиопсию костного мозга и позитронно-эмиссионную томографию, позволило исключить системные проявления мастоцитоза. Заключение. Несмотря на то, что мастоцитоз у детей в основном представлен кожной формой, необходимо проводить тщательное обследование пациента на предмет системного поражения. Антигистаминные препараты в сочетании с топической и/или системной терапией глюкокортикоидами часто эффективны, но полный регресс патологических проявлений заболевания происходит не всегда. При отсутствии или недостаточном терапевтическом эффекте показано использование иных терапевтических опций — таргетных препаратов (ингибиторов тирозинкиназ).

Background. Mastocytosis is a very rare disease with various manifestations, based on abnormal clonal proliferation of mast cells in organs and tissues, such as: skin, bone marrow, lymph nodes, liver, spleen, and gastrointestinal tract. The diagnosis can be established according to clinical manifestations, laboratory, and instrumental data. Darier’s sign and histological examination are crucial for mastocytosis diagnosis. The presented clinical case describes very rare cutaneous form of mastocytosis. Clinical case description. The girl, 2.5 years old, was hospitalized with multiple erythematous papules on her body, face, and limbs. Comprehensive examination, including bone marrow biopsy and positron-emission tomography, allowed us to exclude mastocytosis systemic manifestations. Conclusion. Despite the fact that mastocytosis in children is mostly represented by skin form, it is necessary to perform complex patient examination on any systemic damage. Antihistamines in combination with topical and/or systemic glucocorticoids are often effective, but complete response does not always occur. Implementation of other therapeutic options, such as targeted drugs (tyrosine kinase inhibitors), is suggested In case of no or insufficient therapeutic effect.

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