Café au lait spots are light brown/tan, flat, round or oval lesions with distinct margins ranging in size from a few millimeters to 15- 20 cm, and can occur anywhere on the body. Many or most are present at birth or develop in the first few months of life, however, they may increase in number and size with age.
Café au lait spots are in and of themselves benign lesions, however, they can be associated with some genetic diseases such as neurofibromatosis-1 in which one of the diagnostic criteria is to have six or more café au lait spots greater than 5 mm. Occasionally, families may have autosomal dominant, multiple café au lait spots without other criteria for neurofibromatosis. Large café au lait spots can be seen in McCune-Albright syndrome.
Differential diagnosis
In the newborn, some flat congenital nevi can be confused with café au lait spots. The can be distinguished through biopsy. In addition, café au lait spots can be confused with freckles, lentigines, postinflammatory hyperpigmentation, Nevus of Ito or Ota, linear and whorled nevoid hypermelanosis, and Mongolian spots.
Pathophysiology
Melanocyte activity is increased and subsequently melanin production is increased in the melanocytes and epidermal cells. In contrast with melanocytic nevi, there is no increase in the number of melanocytes. There have been no differences demonstrated between the melanocytes of café au lait spots vs. the melanocytes from normal skin.
Treatment
Treatment is unnecessary; however laser can be used with variable results for cosmetic reasons.