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Café-au-lait macules

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Café-au-lait macules

Also known as: cafe au lait spots, brown birthmarks

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. CALMs are very common and are seen in 10–20% of the normal population. In rare cases, they may be a sign of an underlying genetic condition.

What causes CALMs?

There are no known causes for CALMs, except when associated with genetic conditions such as McCune-Albright syndrome or neurofibromatosis.

Ten to 20 percent of children have a single café-au-lait spot and up to one percent of children have three or more CALMs.

What do CALMs look like?

Café-au-lait spots 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 millimetres to greater than 20 cm in diameter.

CALMs are usually present at birth or appear during early childhood. They enlarge proportionately with overall body growth but remain stable in size during adulthood.

What other problems can occur with CALMs?

There are a number of genetic conditions which feature café au lait spots, the most common of which is neurofibromatosis type I (NF1).

There have been no reports of café-au-lait spots  becoming cancerous.

How are CALMs diagnosed?

Diagnosis is clinical and based on the appearance of the macules.

If there are multiple café au lait macules present, further testing to exclude an underlying genetic disorder may be needed.  In more complex genetic conditions, having 6 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 CALMs treated?

Topical agents will have no effect on the appearance of café-au-lait spots.

CALMs may be treated for cosmetic reasons with pigment-specific lasers such as the Q-switched ruby, Q-switched alexandrite, and Q-switched Nd:YAG lasers. However, multiple treatment sessions are usually required, responses are variable and recurrences are common.

Surgical excision can also be performed in cosmetically acceptable cases.

When CALM spots are associated with neurofibromatosis or another underlying condition, monitoring and referral to a specialist is required.

This information has been written by Dr Davin Lim and Dr Heba Jibreal
Updated 4 October 2016

 

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