Café au Lait Spots: Macules, Causes, Differential Diagnosis, Removal, Pictures

Cafe au Lait Macule
Cafe au Lait Macule

Café au lait is French for Coffee with Milk. It is used to refer to pigmented birthmarks with the color of coffee with milk. Here is more on Causes, Differential diagnoses, treatment, and pictures.

Café au Lait Spots

They are brown spots that appear anywhere on the body. Café au lait spots range in color from light brown to dark brown. Note that color presentation varies with skin tone and color. They appear as coffee with cream in people with light skin or black coffee (darker than surrounding skin) in those with dark skin tones.

Cafe au lait spots
Cafe au lait spots

Cafe au lait spots are evenly colored round or oval patches that range in size from 2mm to 20 cm in diameter. They are permanent and may increase in size, number or darkness throughout childhood

The spots may be flat or raised with distinct edges. The borders may be smooth or irregular.

Other names include;

  • Hypermelanosis
  • Recklinghausen spots
  • Giraffe spots
  • Coast of Maine Spots
  • Café au lait macules

When do cafe au lait spots appear?

These birthmarks are usually present at birth. They can be observed anytime during infancy. In newborns, the spots may be difficult to isolate and see since they are very faint. They increase in size, number or darkness throughout childhood. They usually become obvious within two to three years of age.

Café au lait spots appear in about 10% of the general population. Prevalence varies with race:

  • 3% in Caucasians
  • 4% in Chinese
  • 3% in Hispanics and
  • 18% in African American

Do cafe au lait spots always mean neurofibromatosis?

Cafe au lait spots in neurofibromatosis
Cafe au lait spots in neurofibromatosis

While café au lait spots are harmless, they are may be associated with neurofibromatosis (NF1). They are usually regarded as the earliest sign of neurofibromatosis. According to Medscape, café au lait spots are usually observed in 95% of patients with neurofibromatosis type 1(NF1). This is a genetic disorder that causes abnormal growth of nerve cell which presents as benign tumors on nerves anywhere in the body.

Generally, an increase in the number of about 6 or more is used to diagnose neurofibromatosis.

It is important to note that it is possible to have multiple cafe au lait spots without nf1.


Café au lait spots/ macules are caused by an excessive production of melanosomes by a normal number of pigment producing cells (melanocytes). However, those related to NF1 are caused by an increased proliferation of melanocytes.

Differential diagnosis

Diagnosis is based on clinical presentation.

The presentation and distribution of café au lair spots may be telling of an underlying problem. The number of spots may have a clinical significance. The following syndromes are ruled out.

  • Neurofibromatosis Type 1
  • Neurofibromatosis Type 2
  • Silver Russel Syndrome
  • Bloom Syndrome
  • Watson Syndrome
  • Leopard Syndrome
  • McCune Albright Syndrome
  • Legius Syndrome

Note that the size or shape of café au lait spots does not have any implications with regards to associated syndromes. Another form of diagnosis is genetic testing.

On Face

Café au lait spots on the face may be a cosmetic concern, especially for women. The possibility that the birthmarks may become darker with age may cause low self-esteem in teenage years. Consult a dermatologist for treatment options for large birthmarks on the face.

In Babies

Café au lait spots are usually smaller and faint in babies. They may enlarge and appear darker as the child grows but become less prominent in adulthood.

In Adults

Unless they qualify for examination for NF1, café au lait spots do not require any form of treatment. They grow faint and become almost invisible in adulthood.

Café au Lait Removal- Treatment

The presence of 1 to 3 spots doesn’t require treatment. However, the presence of 6 or more spots especially in children less than 5 years needs to examined for neurofibromatosis and other similar syndromes. Neurofibromatosis is a genetic disorder, family history is very important when examining children with café au lait spots.

Treatment is usually done for cosmetic purposes. It involves removal using lasers. Otherwise, café au lait spots are permanent and persist for life. Note that results on laser treatment are varied and once removed, there may be a recurrence.


There are no reports to indicate whether café au lait spots undergo any malignant change. They may have significant clinical changes but are not fatal. Café au lait spots may be associated with other syndromes. They however, do not produce any morbidity or mortality.


Here are pictures showing different presentations of cafe au lait spots

Cafe au lait macule
Large cafe au lait macule with distinct but irregular borders
Cafe au lait birthmark
Cafe au lait birthmark

Diseases of Pigment:

Café au lair macule:

1 Comment

  1. My daughter is 2 month old born with cafe Au Lait.. on her left cheek.. it doesn’t look good. I want to remove it as soon as possible. I don’t want to give her this bad gift to her. Because ppl are already passing comments. Plz tell me when I can start her treatment.

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