Histiocytosis X

Abstract

The recently suggested term “histiocytosis X” seems preferable to the names formerly used for the disease picture which includes as variants Hand-Schüller-Christian disease, Letterer-Siwe disease and eosinophilic granuloma of skin and bone. The new term represents the complete disease spectrum, describes the basic pathologic alteration, and indicates that the cause is unknown. Histiocytosis X may be acute and disseminated, chronic and slowly progressive, or benign and localized. A proliferative reaction of the reticuloendothelial system is always present.

Treatment of histiocytosis X is empiric. In the cases presented, the authors used x-ray therapy, electron beam therapy from the linear accelerator, nitrogen mustard, antibiotics, and corticosteroids.