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Systemic juvenile xantogranuloma in a child
https://doi.org/10.21508/1027-4065-2025-70-5-108-113
Abstract
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.
About the Authors
I. N. Cherezova
Kazan State Medical Academy
Russian Federation
Russian Federation
420012, Kazan
N. Kh. Gabitova
Kazan State Medical Academy
Russian Federation
Russian Federation
420012, Kazan
I. V. Osipova
Republican Children’s Hospital
Russian Federation
Russian Federation
420138, Kazan
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Review
For citations:
Cherezova I.N., Gabitova N.Kh., Osipova I.V. Systemic juvenile xantogranuloma in a child. Rossiyskiy Vestnik Perinatologii i Pediatrii (Russian Bulletin of Perinatology and Pediatrics). 2025;70(5):108-113. (In Russ.) https://doi.org/10.21508/1027-4065-2025-70-5-108-113
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