Multiple CALMs in a patient with neurofibromatosis.

CAFE AU LAIT MACULE

The cafe au lait macule (CALM) is a uniformly pigmented patch with onset at birth or early childhood.

• Solitary CALMs are common birthmarks and are found in 2.7% of normal neonates
• CALMs often develop in early childhood as well and about 28% in preschool-aged children have at least one.
• Multiple CALMs may be associated with neurofibromatosis, McCune-Albright Syndrome or Legius Syndrome
• The presence of 6 or more CALMs greater than 0.5 cm in prepubertal individuals (15 mm after puberty) is one diagnostic criteria for neurofibromatosis.
• It has been noted however that there are a subset of children, many with fair complexion and red or blond hair, that have an increased number of feathery CALM but are unlikely to develop NF-1.

Clinical

The CALM is a light tan or "coffee with milk" colored patch (color change only, not palpable) that is either congenital or noticed in early childhood. CALM may also be associated with juvenile xanthogranulomas

Typical vs. Atypical CALM

Some have defined two morphologies of CALM--typical and atypical. Typical CALM are ovoid in shape, with even borders, and uniform pigmentation and are more common in NF1. Atypical CALM have ragged/irregular borders and are irregular in shape. Patients with atypical CALM are less likely to develop NF-1 than those with typical CALM.

Treatment

Usually no treatment is done for this benign lesion. The main intervention in a child with multiple CALMs is to exclude neurofibromatosis.

The pigmented laser has been reported to cause significant lightening or clearing although individual results are variable.

A cafe au lait macule to the left and a nevus spilus to the right.

Multiple CALM in a child with neurofibromatosis and juvenile xanthogranulomas.

JXG

A more subtle CALM with some cherry hemangiomas in an elderly man.

RegionalDerm

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