Histiocytosis X with involvement of brain

Abstract

The clinical histories and the autopsy findings of 3 cases of acute disseminated histiocytosis X (Letterer-Siwe disease) are reviewed. Microscopic examination of the central nervous system in all 3 cases revealed scattered foci of proliferating histiocytic cells in the leptomeninges and in the perivascular areas. This “meningo-encephalitic” type of involvement is limited to the temporal lobe in one case while it is generalized in the other 2 cases. A review of the literature discloses only 4 previously reported cases with a similar type of central nervous system involvement. The authors suggest that the routine careful examination of the brain in every case of histiocytosis X coming to autopsy in the future probably will demonstrate that the type of involvement reported here is more common than generally appreciated.