Contributor: Gordon K. Klintworth
Nevoxanthogranuloma (juvenile xanthogranuloma) is a benign self-limiting proliferation of histiocytes presenting in infancy. Although the term xanthogranuloma has been used to describe a xanthoma-like lesion associated with a chronic inflammatory cell infiltration, the lesion is a self-limiting proliferation of histiocytes presenting in infancy. The lesions mainly involve the skin. A common clinical presentation is a spontaneous hyphema from bleeding lesions in the iris. Compared with the iris and eyelids, involvement of the orbit is rare. Touton giant cells and other inflammatory cells infiltrate the iris. Juvenile xanthogranuloma is a type of histiocytoma, but it is not considered as part of histiocytosis X, because the histiocytes lack the characteristic Birbeck granules. Persons with juvenile xanthogranuloma usually has fewer skin lesions than those with xanthoma disseminatum and they tend to be self-limited. The lesions of juvenile xanthogranuloma progress for 1-2 years and then resolve spontaneously.