Introduction

Xanthogranuloma juvenile is the most common type of non-Langerhans cell histiocytosis. Most often it occurs in the form of solitary skin nodules, however, extracutaneous localization of lesions is also possible.

Objective

To present the case of a patient with confirmed juvenile xanthogranuloma, and to review the literature on the subject.

Case report

A 3-month-old boy was admitted to the hospital for diagnostics of skin nodules with concomitant changes in the subcutaneous tissue. Juvenile exanthogranuloma was suspected. The ultrasound scanning of the abdominal cavity revealed numerous focal lesions within internal organs. Based on the histopathological examination of a skin nodule and of a nodule of the subcutaneous tissue in the lumbar region, the diagnosis of multifocal juvenile xanthoganuloma was made.

Conclusions

Juvenile xanthogranuloma usually affects the skin only. In these cases the course of the disease is usually mild, with a tendency to self-healing. In cases with extracutaneous lesions, the course of the disease depends on location of lesions and their effect on function of affected organs. In the case of involvement of vital areas, surgical removal, chemotherapy or immunosuppressive treatment is recommended.