Café au Lait Spots

Café au lait spots or café au lait macules are light brown flat lesions on the skin that are noted at birth in up to 1/3 of newborns.
Most of the time, children will have less than three café au lait spots as an isolated finding. The presence of more than five café au lait or a single, large unilateral café au lait may be indicative of an underlying genetic syndrome.

Pathogenesis ⬆ ⬇

Histologically, there is increased basal cell layer melanin and giant melanosomes.

Clinical Manifestations ⬆ ⬇

Lesions present as light brown, evenly pigmented, round or oval, flat macules or patches that can vary in size from small to very large (Fig. 1.18).
Café au lait spots are more common in children with darker skin color.
May occur anywhere on the body and most children have one to three lesions.
Lesions persist and remain stable over a lifetime.
Six or more café au lait spots should prompt consideration for neurofibromatosis (NF) (see Chapter 11: Neurocutaneous Syndromes). The café au lait spots associated with NF are usually oval with smooth, sharply demarcated borders (“typical” café au lait; Fig. 1.19).
Atypical café au lait spots are larger, have irregular or “smudgy” borders and nonhomogeneous pigment and are less likely to be associated with NF1 (Fig. 1.20; Chapter X).
Large, geographic café au lait spots especially those on the abdomen may be a marker of McCune-Albright syndrome.

Diagnosis ⬆

Differential Diagnosis

Congenital Melanocytic Nevus

Congenital Nevus Spilus (see below)

May initially look identical to a café au lait; over time smaller foci of darker brown nevi will develop within the lighter brown background patch.

Epidermal Nevus (see below)

May initially be flat and light brown but over time become raised and can become warty.
Typically present in a Blaschkolinear pattern.

Becker Nevus

An ill-defined hyperpigmented patch that can resemble a café au lait (Fig. 1.21).
Presents during adolescence; typically unilateral and on the chest.

Management

Patients with café au lait spots noted at birth should have a complete skin examination to determine number of café au lait and the presence of other congenital defects.
No specific treatment is necessary.
Laser therapy, including the Er:YAG, or the Q-switched Nd:YAG, Q-switched alexandrite or ruby, for lightening or removal of café au lait has produced variable results. Most studies show 50% of patients experience total clearance but there is a high relapse rate.
Parents should be reassured of their benign and static nature.