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