| • | eruption of red-brown papules with a predilection for flexural areas (antecubital and popliteal fossae) |
| • | mucosal and visceral involvement (50%) – usually without symptoms |
| • | meningeal involvement – 50% develop a xanthomatous infiltration of the floor of the third ventricle leading to hypothalamic-pituitary axis involvement and secondary diabetes insipidus |
| • | histology – foamy (xanthoma) cells and Touton giant cells |
| • | good prognosis, self-limited |
Think of the three "eruptive histiocyte" disease together:
Generalized Eruptive Histiocytosis
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ßà
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Xanthoma Disseminatum
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ßà
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Eruptive Xanthomas
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non-lipidized histiocytes
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xanthoma cells*
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xanthoma cells*
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Touton giant cells
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Touton giant cells
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normal serum lipids
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normal serum lipids
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increased
triglycerides
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* xanthoma cells = lipidized histiocytes
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.
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