Nevoxanthoendothelioma is a dermatologic and pathologic syndrome occurring almost exclusively in infants or children and characterized by histiocytic tumors in the skin and, occasionally, elsewhere. The name is somewhat of a misnomer, since the lesions are not necessarily congenital nor are they proved to be derived from endothelial cells, but the name has served successfully to distinguish the entity from other xanthomas and is now widely accepted.
The individual lesions of nevoxanthoendotheliomas resemble those of the Christian-Schüller syndrome. Unlike this latter, however, nevoxanthoendothelioma is relatively benign and resolves spontaneously in a matter of months or years. Its etiology is totally obscure, and no treatment has been found that hastens the resolution of the lesions.
Ocular manifestations of nevoxanthoendothelioma were first studied by Fry (1949) in a case reported by Blank et al.1 Subsequently, about nine instances of eye lesions with nevoxanthoendothelioma have been observed and are to be reviewed