The McCune-Albright syndrome.

The McCune Albright syndrome is characterized in complete form by (a) patchy areas of a particular type of progressive bone disease termed polyostotic fibrous dysplasia; (b) multiple areas of cutaneous light brown pigmentation or cafe au lait spots; and (c) autonomous hyperfunction of one or more of the endocrine system, especially the gonads and thyroid. Diagnosis can be made with certainty on the basis of the three main features; it should be considered when only two are present because each of the three principal features has special characteristics seldom encountered except in patients with this disorder. The presence of any one of the features should prompt a search for one or both of the others. Of 97 patients referred for precocious puberty to Rieth et al. (1984), eight proved to have McCune-Albright syndrome; two of the children had the classic triad; in three, bone lesions but no macules were present; in the remaining three, cafe cau lait spots were found but polyostotic fibrous dysplasia was absent.