Café au lait macule (CALM) is a well-defined, evenly pigmented brown macule or patch. The term refers to the characteristic homogeneous color of "coffee with milk" that may be light to dark brown depending on the patient's normal skin pigmentation.
Onset is usually evident in early childhood as a solitary lesion, although they may be present at birth. A single lesion is present in 10%-20% of the US population, and 1% of healthy young adults have up to 3 CALMs. Lesions are more common in individuals of higher phototype (3% of Hispanic and 18% of African American newborns have a CALM). CALMs are located anywhere on the body, although they typically appear on the trunk or lower extremities and rarely on the face. They increase proportionally in size as a child grows and may vary in size (1 to >20 cm), but are usually 2-5 cm in adults and are asymptomatic.
Multiple CALMs should alert the clinician to the possibility of an underlying systemic disease. Many disorders are associated with multiple CALMs (as listed in the Differential Diagnosis section).
Lentigines, ephelides – Typically smaller, more numerous, and seen in sun-exposed areas.
Many syndromes are associated with CALMs. Strongly associated syndromes include:
Neurofibromatosis – Neurofibromatosis type 1 is the most common syndrome associated with CALMs with ≥6 CALMs (≥1.5 cm in postpubertal and ≥0.5 cm in prepubertal individuals). CALMs may also be seen in neurofibromatosis type 2.
Multiple familial café au lait syndrome – autosomal dominant
McCune-Albright syndrome - The CALMs of McCune-Albright syndrome compared with neurofibromatosis are typically fewer in number, irregular in contour ("Coast of Maine" – jagged, versus "Coast of California" – more smooth), larger, darker, and tend to be near midline and follow Blaschko's lines. McCune-Albright syndrome signs include precocious puberty, other endocrinopathies, and polyostotic fibrous dysplasia.
A café au lait macule (CALM) is a well-defined, flat area that is darker in color than the rest of the skin (hyperpigmented). The term refers to the characteristic even (homogeneous) color of "coffee with milk," which may be light to dark brown.
Café au lait macules usually appear first in early childhood, although they may be present at birth. They may be single or multiple, and they increase in size as the person gets older.
Café au lait macules can be indicators of more extensive (multisystem) disease, particularly neurofibromatosis, a condition that is also marked by freckles in the armpit and groin regions, as well as neurofibromas.
Who’s At Risk
Single café au lait macule lesions are present in 10-20% of the US population.
1% of healthy young adults have up to 3 café au lait macules.
Lesions are more common in darker-skinned individuals (3% of Hispanic and 18% of black newborns have a café au lait macule), and these individuals have a darker "espresso" pigmentation, compared to those seen in whites.
Signs & Symptoms
Light-to-dark-brown, well-defined, even-colored, and often oval, flat lesions anywhere on the skin except for the linings (mucous membranes) of body cavities such as the mouth, nose, or genital areas.
When to Seek Medical Care
While café au lait macules are common, see your child's doctor or a dermatologist if he or she has unidentified pigmented lesions.
No therapy is needed for café au lait macules. Lasers may be used for cosmetic concerns, although the color often comes back (recurs) after laser treatment.
Bolognia, Jean L., ed. Dermatology, pp.986-987, 1758-1759. New York: Mosby, 2003.