systemic lipogranulomatous disorder characterized by an infiltration of foamy, lipid-laden histiocytes and giant cells which are often associated with varying degrees of fibrosis. Most commonly patients present characteristic skeletal abnormalities (Erdheim Chester disease). The location and degree of visceral involvement account for the disparate clinical course of the disease. Lung involvement, when present, consists of an interstitial infiltration with lipid-laden histiocytes. A chronic diffuse infiltrative lung disease may result and characteristically HRCT findings include regular interlobular septal thickening, lung cysts and fine honeycombing.
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