- Children 2-10 yo (overlap)
- Initial otitis media, mastoiditis, polyuria, polydipsia
- Triad diabetes insipidus (posterior pituitary), exophthalmos (late), lytic bone lesions
- Cranium most common, “geopgraphic”
- 30% develop skin lesions, “xanthomatous” intertriginous plaques and nodules
- Other systemic involvement possible
- 60% have chronic course, 10% fatal if hepatic or pulmonary disease
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