Café-au-Lait Spots

What are café-au-lait spots?

Café-au-lait (CAL) spots are pigmented birthmarks that appear as patches on your skin with a light to dark brown color that differs from the tone of the rest of your skin. The term “café-au-lait” means “coffee with milk” in French, which refers to the color of the birthmark, comparing it to the creamy tone of a cup of coffee mixed with milk. These birthmarks can range from a few millimeters to more than 20 centimeters in diameter.

CAL spots are present on newborn babies or show up on the skin early during childhood. They can also develop on adults.

What are the different types of café-au-lait spots?

There are two types of café-au-lait (CAL) spots identified by their shape and pattern.

• Coast of California: The coast of California is the most common type of CAL spot. The spot has clear, smooth borders on all sides, similar to the outline of the state of California’s coast in the U.S. There can be a single spot or multiple CAL spots with clearly defined borders.
• Coast of Maine: The coast of Maine is a less common type of CAL spot. The spots appear to have a rough, jagged border similar to the coastal border for the state of Maine in the U.S.

If you have multiple coast of California CAL spots, visit your healthcare provider. Multiple CAL spots could indicate an underlying condition, like neurofibromatosis (NF1) or other genetic syndromes, which affect your skin and nervous system.

Who do café-au-lait spots affect?

Café-au-lait (CAL) spots can affect anyone. The birthmark is congenital (present at birth), although it may be hard to see on a newborn baby’s skin. The spots become more visible with age, especially after two years.

CAL spots are more prevalent among people with darker skin tones, but birthmarks can appear on anyone.

Some CAL spots relate to genetic conditions where they are an inherited trait in your family history. To better understand your risk of having a child with a genetic condition, talk to your healthcare provider about genetic testing.

How common are café-au-lait spots?

Café-au-lait (CAL) spots are common among children. An estimated 10% of the population has at least one CAL spot.

More than six CAL spots are present on an estimated 95% of people diagnosed with neurofibromatosis type 1 (NF1).

How do café-au-lait spots affect my child’s body?

Café-au-lait (CAL) spots are benign (noncancerous), harmless and will not affect your child’s body. If your child has more than six CAL spots, visit your healthcare provider because multiple CAL spots can be a sign of an underlying genetic condition.

What do café-au-lait spots look like?

Café-au-lait (CAL) spots have a distinct appearance on the skin that sets them apart from other birthmarks. Characteristics of CAL spots include:

• Flat spots on the skin, typically on the torso, arms, legs and buttocks.
• Light brown to dark brown color.
• Round or oval shape, between 2 millimeters to more than 20 centimeters in diameter.
• Smooth or rigid border around the spot.
• Spots can grow in size and number with age.
• Spots are painless and don't cause itchiness or other symptoms.

What causes café-au-lait spots?

An increase in the number of skin cells (melanocytes) that produce the protective pigment that darkens skin (melanin) in the thin outer layer of your skin (epidermis) causes café-au-lait (CAL) spots. The reason for the increase in melanocyte cells is unknown.

people diagnosed with neurofibromatosis type 1 (NF1) have CAL spots due to a mutation of the _NF1 gene. The NF1_ gene prevents cells from growing too quickly, which causes melanocytes to produce too much melanin in your skin.

Sometimes the NF1 gene mutation occurs randomly without being present in a person’s family history while others can inherit this condition from their parents if one of their parents has a mutated copy of the NF1 gene (autosomal dominant). Unlike all other autosomal dominant conditions that only need one mutated gene to activate the condition in the child, NF1 does not trigger until later during a person’s lifetime when a genetic mutation of their second copy of the NF1 gene occurs.

How are café-au-lait spots diagnosed?

After your child is born, your child’s healthcare provider will physically examine your baby to make sure they’re healthy. During this exam, they will take note of any birthmarks on your child’s body, including café-au-lait spots and ask you to keep an eye on how many your child has as they grow older. Spots might not be entirely visible on your newborn, but they might become more visible after your child turns two years old or after sun exposure.

What conditions have café-au-lait spots as a symptom?

Multiple (more than six) café-au-lait spots can be a sign of an underlying genetic condition including:

• Neurofibromatosis type 1 (NF1).
• McCune-Albright syndrome.
• Noonan syndrome.
• Watson syndrome.

If you notice your child has more than six café-au-lait spots on their body, visit your child’s healthcare provider.

How are café-au-lait spots treated?

Café-au-lait spots don't need treatment since they are benign (noncancerous) and do not cause symptoms.

If you want to remove your café-au-lait spots for cosmetic reasons, talk to your healthcare provider about laser treatment. With laser treatment, there is a risk of hyperpigmentation, scarring, the spot’s pigmentation might not clear completely or they could return after being removed.

How can I reduce my risk of having a child with café-au-lait spots?

You cannot reduce your risk of café-au-lait spots because there is no way to prevent birthmarks.

For people who have a history of genetic conditions like neurofibromatosis type 1 (NF1) running in their family, talk to your healthcare provider about genetic testing to understand your risk of having a child with the condition before you plan on becoming pregnant.

What can I expect if I have a child with café-au-lait spots?

Café-au-lait (CAL) spots are present from birth and will be with your child for their entire life. The spots may change size and shape over time and could become more noticeable when exposed to the sun. If you see that your child acquires more than six CAL spots on their body, visit your child’s healthcare provider to see if the spots could be a sign of an underlying genetic condition.

When should I see my healthcare provider?

Visit your healthcare provider if you notice your child’s café-au-lait (CAL) spots appear swollen, bumpy or lumpy to the touch or if they increase in size or number. It's common to have a few CAL spots, but more than six spots can be a sign of an underlying condition. Additional symptoms of common underlying conditions that cause CAL spots as symptoms include:

• Speech and language problems.
• Noncancerous tumors under the skin (neurofibromas).
• Freckles under the armpits.
• High blood pressure (hypertension).
• Bone growth problems (scoliosis).
• Attention-deficit/hyperactivity disorder (ADHD).

What questions should I ask my doctor?

• Are my child’s café-au-lait spots a sign of a genetic condition?
• Can I get a genetic test to assess my risk of having a child with a genetic condition?
• What should I do if I notice my child has more than six café-au-lait spots on their body?

Can café-au-lait spots suddenly appear in adulthood?

Yes. Café-au-lait (CAL) spots are most often present on a newborn’s skin but it is common for CAL spots to develop on a person’s skin later in life. It is normal for a person to have a few CAL spots but more than six spots may be a sign of an underlying condition. No matter what age, if you notice you have more than six CAL spots on your body, contact your healthcare provider to examine your spots for an underlying condition.

Can you have café-au-lait spots without having neurofibromatosis?

Yes. It is very common for people to have a few café-au-lait spots on their bodies without having an underlying condition like neurofibromatosis type 1 (NF1). People diagnosed with NF1 usually have more than six café-au-lait spots on their bodies along with additional symptoms related to their diagnosis.

Are café-au-lait spots the same as a rash?

Although they may look like a rash, café-au-lait (CAL) spots are not the result of an allergic reaction. CAL spots differ from a rash based on their light to dark brown color (whereas rashes are pink to red in color) and they don’t create any symptoms like itchiness that you might get from a rash.

A note from Cleveland Clinic

Café-au-lait spots are harmless birthmarks that appear on your child’s skin. Although they are harmless, keep count of how many spots are on your child’s skin because more than six spots could be a sign of an underlying condition. Reach out to your healthcare provider if you notice changes to your child’s birthmarks.