Table of ContentsView AllTable of ContentsWhat Are They?AppearanceCompared to BirthmarksCausesAssociated ConditionsWhen to Seek Medical CareDiagnosisTreatment
Table of ContentsView All
View All
Table of Contents
What Are They?
Appearance
Compared to Birthmarks
Causes
Associated Conditions
When to Seek Medical Care
Diagnosis
Treatment
Cafe-au-laitspots (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.
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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 underlyinggenetic disorder.
Typically, cafe-au-lait spots arecongenital, 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.
Are Cafe-au-Lait Spots Rare?Cafe-au-lait spots are a type 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.
Are Cafe-au-Lait Spots Rare?
Cafe-au-lait spots are a type 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.” 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:
The Shape and Borders of Cafe-au-Lait SpotsThe borders of cafe-au-lait are sometimes compared to the California and Maine coastlines.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.
The Shape and Borders of Cafe-au-Lait Spots
The borders of cafe-au-lait are sometimes compared to the California and Maine coastlines.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.
The borders of cafe-au-lait are sometimes compared to the California and Maine coastlines.
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.
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:
Skin Discoloration Causes and Treatments
What Causes Cafe-au-Lait Spots?
An increase in the number of melanocytes causes cafe-au-lait spots.Melanocytesare skin cells that producemelanin, a pigment that darkens the skin.Having more than six cafe-au-lait spots can signify an underlying genetic condition.
What Is Skin Pigmentation?
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. In addition to cafe-au-lait spots, symptoms include:
An Overview of Eye Tumors
While it’s less frequent, other genetic conditions may be associated with multiple cafe-au-lait spots. 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.
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?
It’s also important to see a healthcare provider if you or your loved one has the following:
If your healthcare provider suspects or diagnoses you or your loved one with NF1, they will want to schedule periodic checkups.
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 suggestgenetic testing.
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 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 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.
6 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Albaghdadi M, Thibodeau ML, Lara-Corrales I.Updated approach to patients with multiple café au lait macules.Dermatol Clin. 2022;40(1):9-23. doi:10.1016/j.det.2021.08.002MedlinePlus.Skin findings in newborns.Gibbs M, Makkar HS.Chapter 22: disorders of hyperpigmentation and melanocytes. In: Neonatal Dermatology (Second Edition). Elsevier; 2008: 397-421.Waldman R, Grant-Kels J.Chapter 10: benign brown-black and pigmented skin growths. In: Waldman R, Grant-Kels J, eds. Dermatology for the Primary Care Provider. Elsevier; 2022:211-219.MedlinePlus.Birthmarks-pigmented.National Institute of Neurological Disorders and Stroke.Neurofibromatosis fact sheet.Additional ReadingAlbaghdadi M, Thibodeau ML, Lara-Corrales I.Updated approach to patients with multiple café au lait macules.Dermatol Clin. 2022;40(1):9-23. doi: 10.1016/j.det.2021.08.002
6 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Albaghdadi M, Thibodeau ML, Lara-Corrales I.Updated approach to patients with multiple café au lait macules.Dermatol Clin. 2022;40(1):9-23. doi:10.1016/j.det.2021.08.002MedlinePlus.Skin findings in newborns.Gibbs M, Makkar HS.Chapter 22: disorders of hyperpigmentation and melanocytes. In: Neonatal Dermatology (Second Edition). Elsevier; 2008: 397-421.Waldman R, Grant-Kels J.Chapter 10: benign brown-black and pigmented skin growths. In: Waldman R, Grant-Kels J, eds. Dermatology for the Primary Care Provider. Elsevier; 2022:211-219.MedlinePlus.Birthmarks-pigmented.National Institute of Neurological Disorders and Stroke.Neurofibromatosis fact sheet.Additional ReadingAlbaghdadi M, Thibodeau ML, Lara-Corrales I.Updated approach to patients with multiple café au lait macules.Dermatol Clin. 2022;40(1):9-23. doi: 10.1016/j.det.2021.08.002
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Albaghdadi M, Thibodeau ML, Lara-Corrales I.Updated approach to patients with multiple café au lait macules.Dermatol Clin. 2022;40(1):9-23. doi:10.1016/j.det.2021.08.002MedlinePlus.Skin findings in newborns.Gibbs M, Makkar HS.Chapter 22: disorders of hyperpigmentation and melanocytes. In: Neonatal Dermatology (Second Edition). Elsevier; 2008: 397-421.Waldman R, Grant-Kels J.Chapter 10: benign brown-black and pigmented skin growths. In: Waldman R, Grant-Kels J, eds. Dermatology for the Primary Care Provider. Elsevier; 2022:211-219.MedlinePlus.Birthmarks-pigmented.National Institute of Neurological Disorders and Stroke.Neurofibromatosis fact sheet.
Albaghdadi M, Thibodeau ML, Lara-Corrales I.Updated approach to patients with multiple café au lait macules.Dermatol Clin. 2022;40(1):9-23. doi:10.1016/j.det.2021.08.002
MedlinePlus.Skin findings in newborns.
Gibbs M, Makkar HS.Chapter 22: disorders of hyperpigmentation and melanocytes. In: Neonatal Dermatology (Second Edition). Elsevier; 2008: 397-421.
Waldman R, Grant-Kels J.Chapter 10: benign brown-black and pigmented skin growths. In: Waldman R, Grant-Kels J, eds. Dermatology for the Primary Care Provider. Elsevier; 2022:211-219.
MedlinePlus.Birthmarks-pigmented.
National Institute of Neurological Disorders and Stroke.Neurofibromatosis fact sheet.
Albaghdadi M, Thibodeau ML, Lara-Corrales I.Updated approach to patients with multiple café au lait macules.Dermatol Clin. 2022;40(1):9-23. doi: 10.1016/j.det.2021.08.002
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