Juvenile xanthogranuloma.
Three infants with juvenile xanthogranuloma are described. Of the two with ocular involvement, one responded to topical corticosteroid and mydriatic therapy and the other to irradiation. The third had skin lesions alone. In the first case new histologic findings were made: lipid vacuoles were observed, by light and electron microscopy, in smooth muscle cells of erector pili and in Schwann cells of small nerves. These findings, along with the detection of lipid vacuoles in mast cells, support the concept that the condition results from local tissue injury that evokes a histioxanthomatous response. Treatment of juvenile xanthogranuloma should be tailored to the individual case and ranges from no active intervention (when there are skin lesions alone or ocular involvement limited to the eyelid or the epibulbar tissue) to a combination of corticosteroid therapy, irradiation and surgery (when there are iris lesions, which rarely resolve spontaneously and may have serious complications). A trial of topical corticosteroid therapy alone in uncomplicated cases is suggested since it was curative in one of the cases presented here.
D003228:Conjunctiva / Q000648:ultrastructure; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007223:Infant; D007498:Iris; D008297:Male; D008854:Microscopy, Electron; D012867:Skin / Q000648:ultrastructure*; D014603:Uveal Diseases / Q000188:drug therapy / Q000209:etiology*; D014617:Vacuoles / Q000648:ultrastructure; D014972:Xanthogranuloma, Juvenile / Q000150:complications / Q000473:pathology*
D005938:Glucocorticoids