By Gary M. White, MD
The cafe au lait macules (CALM) is a uniformly pigmented patch with onset at birth or early childhood.
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 xanthogranuloma (see photos below).
Some have defined two morphologies of CALM, typical and atypical. Typical CALM are found in patients with NF1 and are ovoid in shape, with even borders, and uniform pigmentation. Atypical CALM seen in otherwise healthy individuals have irregular borders. Atypical CALM have less predictive value for NF-1 than typical CAL spots.
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. For example, the Q-switched 755-nm alexandrite laser gave good to excellent results in slightly over half of patients in one study [J Dermatolog Treat. 2012 Dec;23:431-6.]. Recurrences were low. Males responded more favorably than females. In one study, CALMs with jagged or ill-defined borders responded much better than those with smooth or well-defined borers [JAMA Derm 2017;153;1158].
Low-fluence 1064-nm Q-switched Nd:YAG laser therapy afforded good clinical improvement for treating CALMs [JAAD 2015;73;477–483].
Multiple CALM in neurofibromatosis.
A nevus spilus and a cafe au lait spot.
Infant with neurofibromatosis and a juvenile xanthogranuloma.
An isolated cafe au lait macule in an elderly man.
Cafe au lait macules are common in multiple lentigenes syndrome.
CALMs of NF-1 in the same patient may be either uniform in color or variable. JAAD September 2010 Volume 63, Issue 3, Pages 440–447
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