Portal de Periódicos da CAPES

Reticulo-Endotheliosis

1951; Radiological Society of North America; Volume: 57; Issue: 5 Linguagem: Inglês

10.1148/57.5.642

1527-1315

John R. Hodgson, Roger L.J. Kennedy, John D. Camp,

Osteomyelitis and Bone Disorders Research

There is considerable confusion of terminology regarding the group of diseases known as “Hand-Schüller-Christian disease,” “Letterer-Siwe disease,” and “eosinophilic granuloma of bone.” This confusion might be expected as a result of varying concepts of their pathogenesis. The relationship of lipoid to the conditions, which was emphasized by Rowland (1) has been reflected in the widespread use of terms designating the presence of lipoid material in the lesions. Since reticuloendothelial cells also are present, modifications of the terminology to indicate their presence have been proposed. To the words “lipoid” and “reticulo-endothelial,” a third term has been added in an effort to describe another variant of the disease, namely, “granuloma,” indicating chronicity and a tendency to healing with fibrous tissue and collagenization. All possible combinations of these terms may be found in the medical literature, depending on the particular phase of the disease that is prominent in the case or cases reported. None of the designations that have been proposed is entirely satisfactory and we have no new one to suggest. Because of the apparent basic relationship of Hand-Schüller-Christian disease, Letterer-Siwe disease, and eosinophilic granuloma, it would seem that a fairly general term which would be descriptive, inclusive, and yet fairly specific is to be desired. Until this can be found, we shall refer to these diseases as the reticulo-endothelial group and specify the type, as Hand-Schüller-Christian disease, Letterer-Siwe disease, or eosinophilic granuloma, if possible. It may be that “systemic reticulo-endothelial granulomatosis,” as suggested by Wallgren (2) is the best designation for the group, although it does have certain features that make it not entirely satisfactory. Concepts of the pathogenesis of this group of diseases have changed through the years. As more of the aspects of the condition have become evident, emphasis has been placed on different phases. The syndrome of diabetes insipidus, exophthalmos, and defects of the membranous bones was originally described by Hand (3) in 1893 as a tuberculous granuloma of bone with some secondary hypophyseal involvement. In 1915 Schüller (4) reported 2 cases with defects in the membranous bones of the skull, and in 1920 Christian (5) also reported a case with lesions of the skull, but he felt that the disease was related to some pituitary dysfunction. In 1928 Rowland postulated that the lesions in this group of diseases were due to excessive amounts of lipoid in the body fluids. This excess, he believed, caused irritation of the vessel walls and perivascular infiltrations, the latter increasing progressively in size because of “blockage of the reticulo-endothelial system.” Thannhauser and Magendantz (6) later stated that there was no definite proof of the assumption that primary essential xanthomatosis was caused by a disorder of cholesterol metabolism.