Cafe Au Lait Spots

Café Au Lait Spots are hyperpigmented skin lesions that get their name from being similar in color to coffee with milk, the English translation of Café Au Lait. They vary widely in size and color, and may be indicative of neurofibromatosis.

What are Café Au Lait Spots?

Café au lait spots are smooth, irregularly shaped, brownish spots on the skin. Having a few spots is common in adults of all ethnicities. Although the spots themselves are harmless, having several may be a symptom of a more serious condition called neurofibromatosis, also known as “Elephant Man’s disease.” This genetic disease often presents in young children and causes benign tumors to form on the nerves. These tumors may form at any time, anywhere.

Café au lait spots are also symptomatic of other conditions, like Noonan syndrome, Legius syndrome, and several other rarer syndromes. These spots may be observed in infancy, although they are very light at that stage. As a person ages, the spots can grow darker and grow in size. They may appear to be flat birthmarks with distinct edges that are darker than the surrounding skin color. Pigmentation of cafe au lait spots vary from light beige to very dark, almost resembling black coffee. The pigment of the spots are unrelated to the seriousness of the condition, as this is determined by the melanin produced by your skin cells.

Cafe au lait spots may range in size from 5mm to 15mm or larger. Typically, the larger the spots, the more indicative they are of an underlying cause. The spots are usually permanent, although they may grow or increase in size over time. Particularly in children, where spots may be very small in infancy and grow in size until after the age of two.

Symptoms of Café Au Lait Spots

Having a few of these spots is common and they show up in a wide range of people. However, if café au lait spots are accompanied by freckles in the armpit or groin area, bumps under the skin, bumps on the iris of the eye, or impaired vision or hearing, then neurofibromatosis (NF 1) may be likely.

If you think you or your child may have NF 1, you should see a medical doctor for testing and treatment. Diagnosis may involve a visual exam of the cafe au lait spots, an eye exam, ear exam, imaging tests (x-ray, MRI, etc.), and genetic testing. In most cases, an NF 1 diagnosis is made before the age of four and requires at least two of the symptoms. This means that if cafe au lait spots are the only symptom present, it is unlikely they are caused by neurofibromatosis.

Causes of Café Au Lait Spots

The melanin forming cells in the outer layer of our skin are what produce the spots. The cause of the spots vary greatly, however, and can arise from a wide range of causes, both related and unrelated to skin pigmentation. Having six or more spots is indicative of an underlying cause, rather than simply a birthmark. Although NF 1 is the most common underlying cause, there are over a dozen conditions that may cause café au lait spots, including:

  • McCune Albright syndrome
  • Tuberous sclerosis
  • Fanconi anemia
  • Idiopathic
  • Ataxia telegiectasia
  • Bloom syndrome
  • Noonan syndrome
  • Chediak-Higashi syndrome
  • Congenital naevus
  • Jaffe Campanacci syndrome
  • Multiple mucosal neuroma syndrome
  • Silver Russell syndrome
  • Wiskott Aldrich syndrome
  • Maffucci syndrome
  • Basal cell nevus syndrome
  • Benign congenital skin lesions

Almost all of these conditions require multiple symptoms for diagnosis. So, if you only have cafe au lait spots and no other abnormal symptoms, either physically or mentally, the spots are unlikely to have been caused by an underlying condition. Most medical doctors can perform a visual exam of the spots and recommend the next steps for ruling out other diagnoses.

How are Café Au Lait Spots Treated?

Café au lait spots are benign and do not cause any illnesses or problems on their own. They can, however, be removed with a laser by a dermatologist or a plastic surgeon. After being removed, the spots may come back again or be resistant to removal completely. There are many over the counter treatments found online and in stores that may claim to lighten skin spots, typically with some kind of bleaching cream. These treatments are not FDA approved and, in fact, can be very harmful to the skin.

If an infant or child has cafe au lait spots, it's best to have the spots regularly monitored by a doctor, even if they aren't showing signs of an underlying cause, yet. A yearly check-up to monitor potential symptoms of NF 1 is recommended. Some conditions may only present after a certain age in children and the change in size of the spots may provide valuable information to the doctor as to how to diagnose the issue.

Neurofibromatosis does not have a cure, but there are multiple treatments available to manage the symptoms. These treatments include surgery to remove lesions and benign tumors, and implants to improve vision and hearing. If NF 1 causes cancerous tumors, they are treated similarly to other cancer diagnoses. This, however, is rare. Treatment for other syndromes which may have caused cafe au lait spots varies but there are no medically-necessary treatments meant to remove the spots themselves, since the spots are benign.

Café Au Lait Spots Prevention

Cafe au lait spots cannot be prevented. They are harmless and typically do not change much. Those with café au lait spots may choose to wear sunscreen over the affected area to prevent darkening if they wish, however this will not prevent their occurrence, only keep them from changing color.

Many of the underlying conditions which cause cafe au lait spots are also not preventable. Particularly, neurofibromatosis because it is a genetic condition that is hereditary. Many of the causes of cafe au lait spots are genetic in nature, making them impossible to prevent. It is possible, however, to prevent them from becoming darker after they present by protecting darkened spots from sun exposure. Covering spots in loose-fitting clothing to prevent exposure as well as applying sunblock to darker areas will prevent them from becoming more noticeable.

Last Reviewed:
June 19, 2018
Last Updated:
June 13, 2018