Erdheim - chester disease

or lipid granulomatosis, a distinctive form of lipidosis associated with characteristic skeletal abnormalities. Liberation of cholesterol from foam cells leads to cardiac and pulmonary manifestations; in addition, xanthomatous patches may occur on the eyelids. Pyelonephritis has also been reported. Sometimes patients have mild pain over areas of skeletal abnormality. The long bones of the limbs are involved and reveal increase in density, coarsened trabecular pattern, medullary sclerosis, and cortical thickening in the diaphysis and metaphysis; the epiphyses show only minor changes or are spared. Increased accumulation of radioisotope is seen on bone scans.

Erdheim Chester disease may be related in some manner to the histiocytoses, but the precise nature of the relationship is not clear.

DR