Multiple juvenile xanthogranulomas without foam cells and giant cells

A 4-month-old boy had numerous nodular lesions. Microscopic findings revealed a histiocytic proliferation without foam cells and giant cells. Immunohistochemical studies showed that the histiocytes belonged to the monocyte-macrophage system and not to the Langerhans cell system. This case is an unusual variant of juvenile xanthogranuloma that, when congenital, may be difficult to differentiate from Hashimoto-Pritzker disease.