Late-onset, giant juvenile xanthogranuloma

Juvenile xanthogranuloma (JXG) is the most prevalent form of non-Langerhans cell histiocytosis. The typical presentation includes small papules or nodules in infants, often resolving spontaneously within a few years without treatment. Histologically, it showcases lipid-laden macrophages and Touton giant cells. However, atypical cases may manifest as larger lesions in older individuals, lacking foamy histiocytes and displaying increased mitotic activity. It is important to consider JXG as a potential diagnosis for skin lesions before assuming malignancy. Immunohistochemistry markers such as CD163, CD4, and CD31 can be instrumental in confirming the diagnosis. In this report, reviewing the literature, we present one such unique case of JXG.