A Case of Schüller-Christian's Disease under Observation for Nine Years

Abstract

Clinical examples of Schuller-Christian's disease were published by Hand (1893), Schuller (1916) and Christian (1919); but the true nature of the malady was first recognized by Rowland in 1928. It is now well known that the disease is basically a disturbance of cholesterol metabolism. It is a form of xanthomatosis in which the xanthomas develop in various organs, especially the bones of the skull. About 60 cases have been published. The report of the case which follows may be justified on the basis of certain unusual features and the long period of clinical observation. In a considerable group of cases marked variations in the clinical symptoms are encountered, which are largely explainable on the basis of the location and size of the xanthomatous lesions. Henschen recognizes seven clinical groups: (1) Cases with skeletal transformations, exophthalmos and diabetes insipidus (complete triad). (2) Cases with skeletal transformations and exophthalmos but without diabetes insipidus (incomplete triad A). (3) Cases with skeletal transformations and diabetes insipidus but without exophthalmos (incomplete triad B). (4) Cases with skeletal transformations but without exophthalmos and without diabetes insipidus. (5) Cases with diabetes insipidus but without skeletal transformations. (6) Cases with disturbances of growth. (7) Cases of so-called xanthosarcomatosis without skeletal transformations and without diabetes insipidus.