Four cases of juvenile xanthogranuloma are reported, One patient had a
papulonodular lesion at birth and developed additional papulonodules
during the first two years of life, after which spontaneous regression
of the lesions occurred, The other three patients each had a single n
odular lesion, These four cases illustrated the-histologic variants of
juvenile xanthogranuloma, histiocytic cells with an eosinophilic cyto
plasm predominated in cases 3 and 4, foam cells predominated in cases
1 and 2, and very large numbers of multinucleate giant cells were seen
in case 2, Immunohistochemical studies demonstrated expression of CD6
8 and vimentin by the dermal infiltrate; S100 protein was expressed on
ly in case 3, An ultrastructural study was done in case 1 and found th
e main cytoplasmic markers classically present in juvenile xanthogranu
lomas. Our results and the data from the literature emphasize that rig
orous analysis of clinical and pathologic features is essential to the
diagnosis of juvenile xanthogranuloma.