Disseminated Xanthosiderohistiocytosis (Xanthoma Disseminatum)

Abstract

Introduction During the 1930's a group of patients without elevated serum lipids or cholesterol and exhibiting the symptom triad of diabetes insipidus, widespread plaque-like xanthomatous lesions of the skin, and xanthomatous involvement of the mucous membrane of the upper respiratory tract were reported by Finney, Montgomery, and Osterberg.^1-3 These cases were originally presented as "xanthoma multiplex" with unusual findings, but subsequently were called "xanthoma disseminatum." Although exceedingly rare, this entity is quite distinct from either the hypercholesteremic or hyperlipemic xanthomatoses and from reticuloendothelioses such as Hand-Schüller-Christian disease. As implied by the title chosen for this paper, we believe that the term "xanthoma disseminatum" by connoting a disturbance of lipid metabolism does not properly designate the disorder, because the accumulation of lipid in the lesions most likely is secondary to the proliferation of histiocytic elements rather than to a metabolic disturbance. Instead of being related to the other xanthomas, a