Histiocytosis X

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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.

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