Vulvar juvenile xanthogranuloma in a patient with neurofibromatosis type 1: A case report
DOI:
https://doi.org/10.58597/rpe.v5i1.141Keywords:
Juvenile Xanthogranuloma, Girls, Vulva, Non-Langerhans Cell Histiocytosis, Neurofibromatosis Type 1Abstract
Juvenile xanthogranuloma (JXG) is a rare benign disorder that belongs to the group of non-Langerhans cell histiocytoses. It manifests during early childhood as papulonodular lesions, mainly on the head and neck. Although it rarely affects internal organs, evaluating ocular involvement is recommended in extracutaneous cases. The diagnosis is mostly clinical, but it is occasionally confirmed with a skin biopsy. We present the case of a two-year-old girl brought by her mother to the pediatric gynecology service due to a lesion in the vulvar area. This presentation is unusual and highlights the importance of considering JXG in the differential diagnosis of dermatologic lesions in pediatrics. JXG lesions are generally benign and are treated if they cause symptoms or to confirm the diagnosis.
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