Pigmented, lipid-laden histiocytes in the central nervous system in chronic granulomatous disease of childhood.

In addition to granulomas, infiltrates of pigmented, lipid-laden histiocytes are a consistent morphologic finding in visceral organs of the reticuloendothelial system in patients with chronic granulomatous disease. We report a case with widespread meningeal and focal brain stem infiltrates of these distinctive histiocytes. The noninfectious involvement of the central nervous system in chronic granulomatous disease may be greater than previously recognized and related to the role of the CNS in the reticuloendothelial system.