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November 1973

Picture of the Month

Author Affiliations

Boston; Chicago
From the Abraham Lincoln School of Medicine, University of Illinois, Chicago.

Am J Dis Child. 1973;126(5):617-618. doi:10.1001/archpedi.1973.02110190497006

Denouement and Discussion 

Polyostotic Fibrous Dysplasia (McCune-Albright Syndrome) 

Manifestations  Major manifestations include polyostotic fibrous dysplasia, skin pigmentation, and precocious puberty. The lesions are frequently present at birth and usually consist of large brown or café-au-lait areas of pigmentation with irregular borders (coast of Maine), although this is not consistent. The lesions are frequently unilateral, but may be isolated or appear in a linear fashion. They may overlie the areas of bone involvement, but are commonly located on the back, buttocks, and neck.The fibrous dysplasia that is manifested by cystic lesions may affect all bones, but the long bones are most commonly involved. The femur and tibia are most frequently affected, followed by the fibula, pelvis, humerus, radius, and ulna. Unilateral involvement may also occur. Fractures, limp, leg length discrepancy, and overgrowth of the skull and facial bones are commonly present. Adult patients can be relatively short due to early closure of the epiphyses.Precocious puberty occurs more