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Fibrous Dysplasia of Bone

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Abstract

THE significance of “fibrous dysplasia of bone,” a relatively recent addition to medical nomenclature, still remains obscure. The term is used to designate certain specific osseous lesions often subclassified into three main types, depending upon their anatomic distribution and their association with other extraskeletal anomalies. In type I, the monostotic, the lesions are single or multiple but are confined to one bone; in type II, the polyostotic, the lesions involve more than one bone; in type III, the lesions are widely disseminated throughout the entire skeleton, although unilateral preponderance is often seen.

In type III fibrous dysplasia of bone, common accompaniments are cutaneous pigmentation and, especially in females, precocious puberty. Albright and his associates1 were the first to demonstrate this relationship clearly. Their published report in 1937 stimulated interest in this curious anomaly and led to its recognition as an established clinical entity; thus, the disorder is often referred to as “Albright’s brown-spot syndrome.” Other descriptive terms have been used, such as “pseudohypcrparathyroidism” and “osteitis fibrosa cystica without hyperparathyroidism.”

For all three types the current trend, as suggested by Lichtenstein and Jaffe,2 is toward the use of the general heading, “fibrous dysplasia,” since in all three types there is striking roentgenologic and histologic similarity among individual lesions. We shall use this term and shall try to justify its use from the evaluation of our own experiences with the pathologic state it designates.

CLINICAL STUDY

A survey of our records for the past 25 years disclosed 11 cases in which the . . .


 

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