Rare condition thought to be a histiocytosis of the non-Langerhans cell type, with widespread manifestations. It is a systemic lipogranulomatous disorder with infiltration by foamy, lipid-laden histiocytes and giant cells, with or without fibrosis. Musculoskeletal involvement is relatively characteristic.

Dural accumulations, may mimic meningiomas, although the signal characteristics are somewhat different, as the accumulations in ECD are low on T2.

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Credit: Dr Frank Gaillard