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What is Cafe au lait spot?

Cafe au lait spots are macule that are seen in more than 90 percentage of people with Neurofibromatosis Type 1 and 2
  • They appear as light brown round to ovoid macules with smooth borders often located over the nerve trunk with their long axis parallel to the cutaneous nerves
  • It is significant when the number is more than 6 and each more than 1.5 cm in diameter 
Cafe au lait macules are also seen in McCune-Albright syndrome.
They differ from neurofibromatosis in the following
  • They have irregular borders 
  • They are seen in the midline