Cafe-au-Lait Macules

Overview

Cafe-au-lait macules are flat patches of skin that are darker than the surrounding skin, ranging from light tan to medium brown. The name comes from the French for "coffee with milk," which describes their typical color. They have smooth or slightly irregular borders, do not rise above the skin, and can appear anywhere on the body.

A single cafe-au-lait spot is extremely common and is found in many healthy children and adults. On its own it is simply a type of birthmark and is not dangerous. What can be meaningful is the number and size of the spots: having several large ones may be a clue to an underlying genetic condition that a doctor can evaluate.

Symptoms

Cafe-au-lait macules themselves cause no physical symptoms. They are noticed by appearance only and have these features:

• Flat patches that are level with the skin, not raised or scaly
• Uniform light-brown to dark-brown color
• Oval or irregular shape with fairly defined edges
• Size ranging from a few millimeters to several centimeters
• Present from birth or developing in the first few years of life, sometimes becoming more obvious with age or sun exposure

They do not itch, hurt, bleed, or change texture. A spot that becomes raised, itchy, multicolored, or starts changing quickly should be shown to a doctor, as those features are not typical of a cafe-au-lait macule.

Causes

Cafe-au-lait macules form because of a local increase in melanin, the pigment that gives skin its color, within an area of skin. The pigment-producing cells (melanocytes) in the patch make more melanin than the skin around them, producing the darker color.

In most people a spot or two appears for no identifiable reason and has no wider meaning. However, multiple or large cafe-au-lait macules can be a feature of inherited conditions, most notably:

• Neurofibromatosis type 1: A genetic condition in which six or more cafe-au-lait spots, freckling, and benign nerve growths can occur.
• McCune-Albright syndrome: Associated with large, irregularly shaped spots and hormonal and bone changes.
• Other genetic syndromes: Less common conditions can also feature numerous spots.

Risk Factors

• A family history of neurofibromatosis or another condition associated with multiple spots
• Having many spots from early childhood rather than just one or two
• Spots that are unusually large or have very irregular borders
• Other findings such as armpit or groin freckling or soft skin lumps appearing alongside the spots

Diagnosis

Cafe-au-lait macules are usually diagnosed by simply looking at the skin; no test is needed to identify the spot itself. The more important step is deciding whether the pattern of spots suggests an underlying condition.

• Skin examination: A doctor counts the spots and measures the largest ones, since six or more above a certain size raises concern for neurofibromatosis type 1.
• Full-body check: Looking for freckling in the armpits or groin, small skin lumps, or eye findings.
• Referral and genetic evaluation: If a syndrome is suspected, a specialist or genetic assessment may be recommended.

Treatment

Cafe-au-lait macules are harmless and do not require treatment. They are not pre-cancerous and do not need to be removed for medical reasons. Care focuses on two things:

• Reassurance and monitoring: For most people, knowing the spot is benign is all that is needed. A doctor may suggest watching for new spots in childhood.
• Treating any underlying condition: If an associated syndrome is found, care is directed at that condition and its complications, not at the spots themselves.

For cosmetic reasons, some people seek to lighten a spot with laser treatment. Results vary, spots can return, and this is purely elective. Sun protection helps keep spots from becoming more noticeable.

Prevention

Cafe-au-lait macules cannot be prevented because they are present from birth or determined by genetics. There are, however, sensible steps:

• Use sunscreen and sun protection so spots and surrounding skin tan less, keeping the contrast lower
• Note any new spots in a young child and mention them at routine checkups
• If a genetic condition runs in your family, discuss it with a doctor or genetic counselor

When to See a Doctor

See a doctor if your child or you have:

• Six or more cafe-au-lait spots, especially larger ones
• Spots together with freckling in the armpits or groin, or soft lumps under the skin
• A family history of neurofibromatosis or another related condition
• A spot that becomes raised, itchy, multicolored, or changes quickly, which needs evaluation to rule out other skin conditions

These features do not mean something is certainly wrong, but they are worth a professional check.

Related Topics

Frequently Asked Questions

References

1. Mayo Clinic. Cafe-au-lait spots.
2. MedlinePlus, U.S. National Library of Medicine. Cafe-au-lait spots.
3. National Institute of Neurological Disorders and Stroke (NINDS). Neurofibromatosis.
4. American Academy of Dermatology Association. Birthmarks.