McCune-Albright syndrome is classically defined by the clinical triad of fibrous dysplasia of bone, café-au-lait skin spots, and precocious puberty. The dental anomalies include malocclusion, hypomineralisation of the primary dentition, taurodontism, dilaceration, crowding or spacing due to the alveolar bone expanding at a rapid rate.
The disease results from somatic mutations of the GNAS gene, specifically mutations in the cAMP-regulating protein, Gs alpha. The extent of the disease is determined by the proliferation, migration and survival of the cell in which the mutation spontaneously occurs during embryonic development.
