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McCune-Albright syndrome

McCune-Albright syndrome is characterised by the association of:

  • endocrinopathy - precocious puberty
  • polyostotic fibrous dysplasia - more severe than in sporadic cases
  • cutaneous pigmentation - coast of Maine 'cafe au lait' spots

Clinical presentation

Presentation is variable

  • leg pain, limp, pathological fracture: ~ 75%
  • abnormal vaginal bleeding: ~ 25%
  • coast of Maine cafe-au-lait spots
    • predominantly on back of trunk: ~ 30-50%
    • often ipsilateral to bone lesions

Etymology

The condition is named after

  • Fuller Albright - American (US) endocrinologist (1900-1969)
  • Donovan James McCune - American (US) paediatrician (1902-1976)

See also

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