Polyostotic Fibrous Dysplasia With Cutaneous Pigmentation

Introduction FIBROUS dysplasia may be manifested by involvement of only one bone (monostotic), more than one (polyostotic), or by McCune-Albright syndrome. The original descriptions of McCune-Albright syndrome in this country consisted of café au lait spots, polyostotic fibrous dysplasia, and sexual precocity.1-3There are a number of good reviews describing the clinical, radiological, and pathological aspects of this syndrome to which the reader is referred.1-8In order to make this diagnosis, the classical triad should be present although some investigators5,6do not consider sexual precocity to be necessary. Lichtenstein,9in 1938 and again in 1942 in collaboration with Jaffe,10stressed the pathological aspects of the bony abnormalities of the disease and termed the true nature of these as a fibrous dysplasia. A 6-year-old Negro boy was admitted to the Pediatric Service of the Boston City Hospital, presenting with polyostotic fibrous dysplasia and café au lait