What Are Cafe-au-Lait Spots?

Cafe-au-lait spots (CALS) are brown spots on the skin. They are a type of birthmark, meaning part of the skin has a different color from the rest. 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. Multiple (especially more than six) cafe-au-lait spots could indicate a genetic disorder. 

This article reviews cafe-au-lait spots, what they look like, associated conditions, when to see a healthcare provider, diagnosis, and treatment.

What Are Cafe-au-Lait spots? 

Cafe-au-lait spots are a type of birthmark. They are flat, painless, benign (noncancerous) hyperpigmented (darker than surrounding skin) patches on the skin. Having a few spots is common and normal. However, having multiple spots, especially more than six, may indicate an underlying genetic disorder.

Typically, cafe-au-lait spots are congenital, meaning they are present at birth, but they may also appear over time or become more visible after sun exposure. Sometimes they fade with age, but usually they're present throughout life.

What Do Cafe-au-Lait Spots Look Like?

"Cafe-au-lait" means "coffee with milk." These spots got their name from their typical color, which often resembles the color of coffee with added milk, but the shade of brown can vary from light tan to dark brown. 

The following are characteristics of cafe-au-lait spots:

• Light or dark brown in color (different from the rest of your skin tone) 
• Flat
• Round or oval shaped
• Smooth and well-defined borders (smooth is typical, but a small percentage are jagged) 
• Often get darker with sun exposure
• Vary in size (from a few millimeters to 20 centimeters, or nearly 8 inches, in width)
• Typically located on the chest, back, arms, legs, and buttocks
• May grow in size and number with age
• May increase in number over time

The Difference Between Cafe-au-Lait Spots and Other Birthmarks

Cafe-au-lait spots are a type of birthmark, a skin marking typically present at birth. Birthmarks are common and also include:

• Stork bites and angel kisses: These are tiny red marks on the face and back of the neck. 
• Port-wine stains: These red or purple marks typically appear on the face and neck, but they can be anywhere on the body. 
• Congenital nevi: Congenital nevi are moles. They occur when melanocytes (pigment cells) grow in clusters. 
• Hemangiomas: These are deep-red markings that may be slightly raised. They are also called strawberry birthmarks. 
• Dermal melanocytosis: These flat, blue-gray, or brown spots are typically on the buttocks or back. 

What Causes Cafe-au-Lait Spots?

An increase in the number of melanocytes causes cafe-au-lait spots. Melanocytes are skin cells that produce melanin, a pigment that darkens the skin. Having more than six cafe-au-lait spots can signify an underlying genetic condition.

Associated Conditions

Neurofibromatosis type 1 (NF1) is the most commonly associated genetic disorder. NF1 affects about 1 out of every 4,000 babies.

Those with NF1 have a genetic mutation 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, the mutation can 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. In addition to cafe-au-lait spots, symptoms include:

• Tumors, including optic gliomas (on the optic nerve of the eye) and neurofibromas (tumors around nerves)
• Freckles in the armpits or groin areas (usually appear around 3 to 5 years old)
• Growths on the iris of the eye (called Lisch nodules or iris hamartomas)
• Bone deformities, especially in the eye socket or the tibia (shin)
• Larger than normal head circumference 
• Shorter than average stature
• Scoliosis (abnormal curving of the spine)
• Behavioral and learning problems

While it’s less frequent, other genetic conditions may be associated with multiple cafe-au-lait spots. Some of these include:

• Watson syndrome
• McCune-Albright syndrome 
• Tuberous sclerosis
• Bloom syndrome
• Hunter syndrome

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. 

It’s also important to see a healthcare provider if you or your loved one has the following:

• Six or more cafe-au-lait spots
• A family history of neurofibromatosis type 1 (NF1) 
• Other symptoms of NF1 

If your healthcare provider suspects or diagnoses you or your loved one with NF1, they will want to schedule periodic checkups. 

Diagnosis

Healthcare providers use physical examinations to diagnose cafe-au-lait spots. They may use special lamps to look at these areas. Your healthcare provider may also evaluate your eyes (or your child’s eyes) and ask about possible symptoms of NF1, including freckles under the arms or in the groin area. If they suspect NF1, they may suggest genetic testing.

Treatment

Since these spots are benign and don’t cause pain, itching, or other symptoms, there is typically no need for treatment. If you or a loved one feels self-conscious about cafe-au-lait spots, you can try makeup to cover them. 

Laser therapy may 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 type 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 type 1 (NF1). If your provider suspects a genetic disorder, they may suggest genetic testing.

A Word From Verywell 

Having birthmarks, including a few cafe-au-lait spots, is common and may appear at birth or later in childhood. However, it’s a good idea to reach out to your healthcare provider if new spots occur or if you or your loved one has more than six. Having multiple spots could be a sign of an underlying genetic disorder.