Fulminant normocholesteremic Xanthomatosis (Histiocytosis X)

Abstract

Advancing medical knowledge has not infrequently changed our interpretation of clinical syndromes and diseases. On one hand, well-defined symptom complexes, once thought to represent specific diseases, were found to be caused by more than one pathological process and, conversely, different clinical syndromes were revealed as different phases and manifestations of one and the same nosologic entity. Thus, conditions as different in their clinical manifestations as Hand-Schüller-Christian disease, Letterer-Siwe disease, and eosinophilic granuloma of bone, each once described as disease entities, are now thought by many to represent merely the acute or chronic and the localized or disseminated forms of the same basic pathologic process.* This change in concept came about with the better understanding of the morphologic picture of these conditions but also with the ever-increasing number of instances in which features of more than one of these diseases occurred simultaneously or subsequently in the same patient. Hand-Schüller-Christian disease seemed