Cafe-au-laitspots (CALS) are brown spots on the skin.

These spots are often present at birth but may appear or become more visible with age.

Having a few of these spots is common and harmless.

Close-up of a woman’s thigh with a cafe-au-lait spot/birthmark.

Victor Manuel Mulero Ramirez / Getty Images

Multiple (especially more than six) cafe-au-lait spots could indicate a genetic disorder.

Victor Manuel Mulero Ramirez / Getty Images

What Are Cafe-au-Lait Spots?

Cafe-au-lait spots are a key in of birthmark.

Having a few spots is common and normal.

However, having multiple spots, especially more than six, may indicate an underlyinggenetic disorder.

Are Cafe-au-Lait Spots Rare?

Cafe-au-lait spots are a key in of birthmark.

Having a few (one to three) spots is relatively common.

Between 25% to 36% of people have them.These numbers are equal among sexes.

Multiple spots, especially more than six, are rare and may indicate an underlying genetic disorder.

What Do Cafe-au-Lait Spots Look Like?

“Cafe-au-lait” means “coffee with milk.”

Spots with a border that resembles the coast of California are the most common.

They have well-defined, smooth borders on each side.

Spots that resemble the coast of Maine are less common.

Their borders are rough and jagged.

Birthmarks are common and also include:

What Causes Cafe-au-Lait Spots?

Associated Conditions

Neurofibromatosistype 1 (NF1)is the most commonly associated genetic disorder.

NF1 affects about 1 out of every 4,000 babies.

Those with NF1 have ageneticmutation that causes melanocytes to make too much melanin, causing dark spots.

About 50% of the time, this is inherited or passed down from parents.

However, themutationcan also occur randomly without a family history.

The reason for this random mutation is unknown.

NF1 causes a variety of symptoms that can change throughout life.

Sometimes a diagnosis is not made until older childhood because the symptoms may not appear immediately.

Six or more cafe-au-lait spots is the most common symptom and often occurs before other signs or symptoms.

NF1 can affect the skin, nerves, bones, and eyes.

Some of these include:

Do Cafe-au-Lait Spots Always Mean Neurofibromatosis?

Having cafe-au-lait spots does not always mean that you or your loved one hasneurofibromatosis.

However, having six or more spots increases the risk.

Almost 60% of those with six or more cafe-au-lait spots develop other features of neurofibromatosis.

When Should I Worry About Cafe-au-Lait Spots?

While birthmarks, including cafe-au-lait spots, are common, they should be evaluated by a healthcare provider.

If they were present at birth, your healthcare provider most likely noted them.

They also can develop or become more apparent at an older age.

Diagnosis

Healthcare providers use physical examinations to diagnose cafe-au-lait spots.

They may use special lamps to look at these areas.

If they suspect NF1, they may suggestgenetic testing.

Laser therapymay be an option for those who want to remove the cafe-au-lait spots for cosmetic reasons.

Laser therapy results are not always consistent, though, and it can be uncomfortable or cause scarring.

Providers typically reserve this treatment for those whose spots cause emotional distress or disfigurement.

Summary

Cafe-au-lait spots (CALS) are a throw in of birthmark.

They are benign (noncancerous), hyperpigmented (darker than surrounding skin) flat spots.

Having a few is common and normal.

There is no special tool used to diagnose cafe-au-lait spots.

Your healthcare provider can make the diagnosis based on a physical examination.

Because these spots are painless and harmless, treatment is cosmetic and optional.

Multiple cafe-au-lait spots, especially more than six, can be associated with a genetic disorder.

The most commonly associated genetic condition is neurofibromatosis jot down 1 (NF1).

If your provider suspects a genetic disorder, they may suggest genetic testing.

Having multiple spots could be a sign of an underlying genetic disorder.

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