A 17-month-old boy presented with a one-year history of asymptomatic, multiple yellow-orange
papules and nodules that hadgradually increased in number and size. Physical examination
revealed numerous, discrete, yellowish-orange papules and nodules measuring 1-6 mm
in diameter on the head, face (Figures 1 and 2), neck and upper back. He had no signs of extracutaneous involvement. Laboratory
investigations, including complete blood count, serum lipid levels, renal panel and
liver tests, were all within normal range. Chest radiography, abdominal ultrasound
and ophthalmolgical examination revealed no abnormalities. Skin biopsy taken from
his left forehead showed foamy histiocytes admixed with many Touton giant cells in
the dermis (Figure 3) which were positive for CD68 and negative for S-100 protein and Langerin, thus confirming
a diagnosis of juvenile xanthogranulomas (JXG). The patient did not receive further
treatment, and was scheduled for annual follow-up. After 4 years of follow-up, most
of his skin lesions regressed only leaving slight scar and hyperpigmentation, without
internal organs affected.

Figure 1Numerous, discrete, yellowish-orange papules and nodules measured 1-6 mm in diameter
on the head, face.

Figure thumbnail gr2

Figure 2Numerous, discrete, yellowish-orange papules and nodules on the face (close-up view).

Figure thumbnail gr3

Figure 3Foamy histiocytes admixed with many Touton giant cells in the dermis (hematoxylin-eosin
stain, 400× original magnification).

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