| • | benign self-limiting disease of infants and children |
| • | both cutaneous and visceral lesions disappear spontaneously within 3-6 years |
papular form:
| • | most common extracutaneous manifestation: ocular involvement |
| • | iris; usually unilateral; may lead to hyphema or glaucoma |
| • | eye exam if <2 years old with multiple JXG’s |
| • | hyphema from a JXG on the eye (mistaken for retinoblastoma) |
| • | benign cephalic histiocytosis – located only on head and neck; infiltrate lacks multinucleated giant cells and foamy cells |
| • | generalized eruptive histiocytosis – lipidation of the cells never occurs |
| • | self healing reticulohistiocytosis – the cutaneous lesions persist for only the first few months of life |
| • | tuberous xanthoma – appears only in hyperlipidemic state |
| • | nodular forms of histiocytosis X – differentiated by histologic, immunocytochemical, and ultrastructural characteristics |
nodular form:
| • | “classic ddx”: solitary mastocytoma, Spitz nevus, JXG |
histology:
| • | foamy cells and Touton giant cells |
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