What are Café Au Lait Spots

What are Café Au Lait Spots?

These spots are light brown or tan, flat patches on the skin that often resemble the color of coffee with milk. Read on to learn why these spots are important.

Café-Au-Lait Spots

Café au lait spots or macules (CALS or CALM) are flat, colored spots on the skin. They are commonly referred to as “birthmarks,” but are often not present at birth. The name café-au-lait spot is derived from the French term for coffee (café) with milk (lait) because they usually have a light brown color. These spots are always darker than the surrounding skin, regardless of ancestry or race.

Many children who are evaluated for multiple skin spots do not receive a definitive answer at their first evaluation. This does not necessarily mean an underlying condition does not exist; it simply means a diagnosis cannot yet be confirmed. For many conditions associated with skin spots, features develop over time, so ongoing follow-up is necessary.

Three close-up images of skin, each showing flat, light brown patches of varying sizes and shapes on different skin tones.

Why are Café Au Lait Spots Important?

Approximately 10% of the general population has one or two café au lait spots. However, having six or more café au lait spots (referred to as multiple CALS) is rare. Multiple café au lait spots alone do not lead to any health problems but may be associated with a number of different conditions that could cause other medical issues. Therefore, your physician may monitor your child and be suspicious in certain situations, such as if the number of spots exceeds five, additional spots appear over time, or your child has other physical, medical, or developmental concerns.

The most common condition associated with multiple CALS is called neurofibromatosis type 1 (NF1).

Other conditions may look like NF1 so it is important to see a specialist who can make an accurate diagnosis.

Need Help Finding a Specialist?

Search for a clinic on our Find a Doctor page at the button below.

Find a Doctor

My Child Has Café Au Lait Spots. What are Other Symptoms (or Manifestations) of NF1?

NF1 can manifest very differently from patient to patient, and no one person will have all of the possible symptoms of NF1. The severity of NF1 ranges from extremely mild cases to more severe cases, in which one or more serious manifestations may develop.

There is no way to predict who will have a mild case and who will develop more serious symptoms, but the majority of those with NF1 will have minor cases (60%).

Read about NF1 Symptoms

Can Multiple Café Au Lait Spots (CALS) be Caused by Something Other Than NF1?

Although NF1 is the most common cause of multiple CALS, they are also a feature of other conditions including Legius syndrome, Noonan syndrome with multiple lentigines (formerly called LEOPARD syndrome), chromosome abnormalities, McCune-Albright syndrome, NF2-related schwannomatosis, and others. Each of these conditions has features that overlap with NF1, but other features that distinguish them. Rarely, multiple CALS can be seen as an isolated feature and not associated with an underlying condition. A specialist in NF is often required to determine if multiple CALS are isolated or caused by a genetic condition.

Resources

Possible NF1: What are Café Au Lait Spots

An informational flyer about café-au-lait spots, featuring a child smiling in the top right corner and detailed text explaining their significance and guidance for families.

An informational handout about café au lait spots and a possible diagnosis of neurofibromatosis type 1 (NF1).

View and Download

Café Au Lait – Children’s Picture Book

Illustrated book cover titled "Café Au Lait: A Story of NF1 and My Special Spots" by Jessica M. Boehman, featuring a child examining spots on their abdomen.

Available for purchase at the CTF Store, this 36-page picture book tells the story of Enzo and his café au lait spots.

Purchase Your Copy

Two pairs of hands hold a red heart-shaped object together. One person wears a white long-sleeved garment, possibly a lab coat, and the other has bare arms.

Newly Diagnosed or Waiting for a Diagnosis?

Getting an NF diagnosis, or waiting for a diagnosis, can be overwhelming. The most important thing to remember is that you are not alone. We suggest starting your journey with these resources.

Newly Diagnosed Understanding NF

A paper airplane flies away from an envelope icon with charts, trailing a dotted line.

Sign Up for our Newsletter

Join our mailing list for news, exclusive resources, events and opportunities.

"*" indicates required fields