The McCune-Albright syndrome is characterized by the triad of localized bone lesions, termed polyostotic fibrous dysplasia, cafe-au-lait pigmentation of the skin, LHRH-independent sexual precocity, and autonomous hyperfunction of multiple
endocrine
systems. A somatic activating point mutation in the gene for the alpha-subunit of the G-protein in the affected has been claimed to be the underlying defect.
The authors report a 13-year-old girl patient showing facial deformity due to polyostotic fibrous dysplasia of the left frontal and sphenoid bones, LHRH-independent sexual precocity, cafe-au-lait skin pigmentation of the left lateral surface of
the
upper humerus ;with literature review.
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