Last updated: January 2024
What are café-au-lait macules?
Café-au-lait macules (CALMs) are benign well-circumscribed, flat, uniformly light to dark brown spots with a characteristic ‘coffee-with-milk’ colour.
Who gets café-au-lait macules?
CALMS are very common and are usually present at birth or appear during early childhood. About 10 – 20% of children have a single café-au-lait spot and almost 1% of children have three or more CALMs.1
What causes café-au-lait macules?
There are no known causes for CALMs, except when associated with genetic conditions such as McCune-Albright syndrome or neurofibromatosis.
What do café-au-lait macules look like?
CALMs contain more melanin (pigment) than normal skin. They are uniform, tan to dark brown flat spots that may be located anywhere on the body except the mucous membranes. They can range from a few mm to greater than 20 cm in diameter.
They enlarge proportionately with overall body growth but remain stable in size during adulthood.
How are café-au-lait macules diagnosed?
CALMs are diagnosed clinically and based on the appearance of the macules.
If there are multiple CALMs present, further testing to exclude an underlying genetic condition may be needed. In more complex genetic conditions, having six or more CALMs larger than 5 mm in diameter before puberty, or larger than 15 mm after puberty may be an important clinical feature.
How are café-au-lait macules treated?
There is no medical treatment required for CALMs.
However, CALMs may be treated for cosmetic reasons with pigment-specific lasers, including:
- Q-switched Nd:YAG laser, Q-switched Alexandrite laser or Q-switched ruby
- Picosecond lasers
Results are variable and recurrences are common. Surgical excision can also be performed in cosmetically acceptable cases.
When CALMs are associated with neurofibromatosis or another underlying condition, monitoring and referral to a specialist is required.
What is the likely outcome of café-au-lait macules?
CALMs continue to persist without treatment. Individuals who respond well to initial treatment often have low recurrence rates.