Polyostotic Fibrous Dysplasia: A Defense of the Entity

IN 1937 and 1938 the author and his colleagues published two papers (1, 2) in which they described the clinical findings of 7 patients with a bizarre syndrome characterized by “osteitis fibrosa disseminata, areas of pigmentation, and precocious puberty in females.” Since that time some authors (3,4,5,6) have been so flattering to the present author as to publish similar cases under the heading of “Albright's Syndrome” others (7, 8) have questioned the entity of the syndrome. It is the purpose of this paper to defend the entity; a few words pertaining to terminology will be added. For the time being let us call the condition under discussion, “Syndrome X.” Snapper (7) feels that Syndrome X is a form of Hand-Schüller-Christian's Disease (xanthomatosis) which he prefers to call “lipoid granulomatosis” and which is closely akin to, if not identical with, eosinophilic granulomatosis. He points out that, whereas the classical case of lipoid granulomatosis is characterized by multiple round defects in the skull, exophthalmos, diabetes insipidus, and other pituitary signs, there need be no cranial or hypophyseal localization; he states that it is in such cases that the condition “is usually not recognized and goes under a different name.” At this point he cites the cases described by the author. To be sure, in the summary he is less dogmatic when he states that “it seems reasonable to conclude that at least part of the cases of osteitis fibrosa disseminata… may well belong to the group of lipoid granulomatosis of the bones without cranial hypophyseal localization… ”