perinatologyРоссийский вестник перинатологии и педиатрииRossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics)1027-40652500-2228Ltd. “The National Academy of Pediatric Science and Innovation”10.21508/1027-4065-2025-70-5-108-113perinatology-2275Research ArticleКЛИНИЧЕСКИЕ СЛУЧАИCLINICAL CASESСистемная ювенильная ксантогранулема у ребенкаSystemic juvenile xantogranuloma in a childhttps://orcid.org/0000-0001-8961-0996ЧерезоваИ. Н.CherezovaI. N.

Черезова Ирина Николаевна — к.м.н., доцент кафедры госпитальной педиатрии.

420012, Казань, ул. Бутлерова, д. 49

420012, Kazan

https://orcid.org/0000-0002-8375-0565ГабитоваН. Х.GabitovaN. Kh.

Габитова Наиля Хусаиновна — к.м.н., доцент, кафедры госпитальной педиатрии.

420012, Казань, ул. Бутлерова, д. 49

420012, Kazan

https://orcid.org/0000-0001-9833-5156ОсиповаИ. В.OsipovaI. V.

Осипова Ильсия Вагизовна — зав. отделением онкогематологии.

420138, Казань, Оренбургский тракт, д. 138

420138, Kazan

ФГБОУ ВО «Казанский государственный медицинский университет» Минздрава РоссииРоссияKazan State Medical AcademyRussian FederationГАУЗ «Детская республиканская клиническая больница»РоссияRepublican Children’s HospitalRussian Federation202514112025705108113Copyright © Ltd. “The National Academy of Pediatric Science and Innovation”, 20252025Ltd. “The National Academy of Pediatric Science and Innovation”Ltd. “The National Academy of Pediatric Science and Innovation”https://www.ped-perinatology.ru/jour/about/submissions#copyrightNoticehttps://www.ped-perinatology.ru/jour/article/view/2275

Ювенильная ксантогранулема — редкая, но распространенная форма нелангергансоклеточного гистиоцитоза, которая поражает младенцев до двух лет жизни. У большинства детей заболевание ограничивается кожей, проявляясь красными или желтыми узелками диаметром 0,5–1,0 см, одиночными или множественными, возникающими на волосистой части головы, лице, шее и туловище. Элементы могут спонтанно исчезать в течение нескольких месяцев или нескольких лет. Большинство пациентов с кожной формой ювенильной ксантогранулемы не нуждаются в лечении. Системное поражение наблюдается у пациентов с множественными кожными узелками и может сопровождаться угрожающими жизни симптомами с уровнем смертности около 4% и редко разрешается спонтанно. В статье представлено клиническое наблюдение нетипичного течения системной ювенильной ксантогранулемы с поражением ЦНС, кожи, легких, печени, мочевого пузыря, матки, влагалища. Дана характеристика клинических и гистологических особенностей заболевания, показаны различные подходы к таргетной терапии ингибиторами BRAF и МЕК.

Juvenile xanthogranuloma is a rare but common form of non-Langerhans cell histiocytosis that affects infants under 2 years of age. In most children, the disease is limited to the skin, manifested by red or yellow nodules 0.5–1.0 cm in diameter, single or multiple, arising on the scalp, face, neck, and trunk. Elements may spontaneously disappear within several months or several years. Most patients with cutaneous juvenile xanthogranuloma do not require treatment. Systemic involvement is observed in patients with multiple cutaneous nodules and can be accompanied by life-threatening symptoms with a mortality rate of about 4% and rarely resolves spontaneously. The article presents a clinical observation of an atypical course of systemic juvenile xanthogranuloma with involvement of the central nervous system, skin, lungs, liver, bladder, uterus, and vagina. The clinical and histological features of the disease are characterized, and various approaches to targeted therapy with BRAF and MEK inhibitors are shown.

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