Café Au Lait Spots

Café Au Lait Spots

Whether found as a single lesion or multiple spots in a child, café au lait spots concern many parents, especially once they’ve read up about them and realized what kind of diseases are associated with them.
But café au lait spots do not have to be a part of a systemic disease. Sometimes they are found as isolated lesions, and do not carry any clinical significance.
So, how can we distinguish between café au lait spots that do have a clinical significance and those that don’t?
Professor Danny Ben Amitai has written a post to address all your questions! Continue reading to learn more…

What are café au lait spots?

These are flat areas of brownish coloured skin, a uniform light brown – see the image attached. In darker skinned individuals, the spots can be a darker brown or even closer to black.
These spots have clear margins but they are not always have regular margins.
They vary in size and can be anywhere from very small to large, covering a large part of the skin they’re on (for example, the back or a limb).
These spots typically increase as the child grows and do not disappear in adulthood. When examining these spots microscopically, you see a large amount of melanin pigment without too many melanocytes.

café au lait spots2

How common are café au lait spots?

About 10% of healthy, light-skinned children have 1-3 café au lait spots. In darker-skinned children, about 25% of children will have at least 1 spot.
These spots are either visible already at birth or develop during childhood.

What should I keep in mind when I notice a café au lait spot?

Most children who have café au lait spots are healthy, but having many of these spots can be a manifestation of a systemic disease, the most common one being Neurofibromatosis type 1.

What is Neurofibromatosis type 1?

This is a multi-organ condition that is autosomal dominant and has a prevalence of 1 in 3,000 people. In about 40% of the cases the clinical manifestations of the disease are present already at birth. A number of café au lait spots is the most common sign present at birth.
In children, one of the diagnostic signs for neurofibromatosis type 1 are 6 café au lait spots, each larger than 5mm in diameter. Adults will have 6 spots that are all larger than 15mm in diameter.

What should I do if I notice a café au lait spot on my child’s skin?

First stop and take a deep breath. Most of the time, this is a spot that has no clinical significance. Afterall, as I mentioned above, about 10% of healthy light-skinned children will have up to 3 of these spots. However, if you notice several of these spots on your child’s skin, it is important to see a dermatologist and undergo a thorough skin examination, to assess whether your child has any more signs that raise concern for neurofibromatosis.

In summary, this is a very common skin lesion seen in healthy children. When multiple spots are seen in a child or there are additional findings, it is important to see an experienced dermatologist for a thorough examination.

For comments and questions, please register

Scroll to top