
What causes café-au-lait macules?
- The epidermal melanocytes of an isolated café-au-lait macule have excessive numbers of melanosomes ( intracellular pigment granules ). ...
- The café-au-lait macules associated with NF type 1 and Leopard syndrome have increased proliferation of epidermal melanocytes (epidermal melanocytic hyperplasia ).
- A café-au-lait macule is not classified as a congenital melanocytic naevus.
What do café-au-lait spots indicate?
Although these colored spots on the skin can be harmless, having six or more café au lait spots with freckles under the arm or around the groin could indicate an underlying genetic problem called neurofibromatosis type 1. This is a disorder that can affect the skin, nerves, and eyes.
Do café-au-lait spots always mean neurofibromatosis?
Café au lait spots (CALS) are extraordinarily common; six or more lesions (at least 0.5 cm prior to puberty or 1.5 cm post puberty) are considered one of the criteria for diagnosing neurofibromatosis type I (NF1), and are also seen in a variety of other syndromes.
How common are café-au-lait birthmarks?
Approximately 10% of the general population has one or two café-au-lait spots. However, having more than 5 café-au-lait spots (referred to as multiple CALS) is rare.
At what age do café-au-lait spots appear?
I find that six or more café-au-lait spots are usually visible by around 2 years of age, and new spots do not usually appear after that time, though spots tend to tan upon sun exposure, so may become more distinct with time.
Should I worry about café-au-lait spots?
Café-au-lait spots are harmless birthmarks that appear on your child's skin. Although they are harmless, keep count of how many spots are on your child's skin because more than six spots could be a sign of an underlying condition. Reach out to your healthcare provider if you notice changes to your child's birthmarks.
At what age do neurofibromas develop?
These tumors typically first appear by age 6, rarely in late childhood and adolescence, and almost never in adults. Although they can affect vision, most do not become symptomatic.
What can be mistaken for café-au-lait?
Café-au-lait macules have a distinctive appearance and are usually easily recognizable on clinical examination, although they may be confused with postinflammatory hyperpigmentation, melasma, or melanocytic nevi.
Do café-au-lait spots run in family?
Multiple café-au-lait spots have been observed in successive generations of several families without any other manifestations of neurofibromatosis (NF) or any other systemic disorder. The café-au-lait spots in these families segregate as an autosomal dominant trait.
What age is NF1 diagnosed?
Neurofibromatosis 1 (NF1) is usually diagnosed during childhood. Signs are often noticeable at birth or shortly afterward and almost always by age 10. Signs and symptoms are often mild to moderate, but can vary in severity.
How do I know if my baby has neurofibromatosis?
Light brown spots on the skin called café-au-lait spots. These are the most common signs of NF, and they often appear at birth or in the first years of life. They're harmless, but if your child has more than six, she probably has NF1. Freckles in the armpits or groin area also are signs of NF1.
Does NF1 get worse with age?
Learning and behaviour problems in NF1 are not progressive, that is they do not get worse over time. In fact, they can usually be improved with appropriate help. NF1 is caused by a change in the structure of a gene. Each person has about 30,000 genes in their body.
Can NF1 go away?
Adulthood: It is impossible to predict the course of NF1 in anyone with the disorder. Manifestations of neurofibromatosis generally do not disappear once they develop, although cafe-au-lait spots sometimes fade in later life.
How big are café au lait spots?
The size of café au lait spots can also vary. Spots can be as small as a half centimeter. The spots are usually present at birth but may develop later in life.
What is a café au lait?
Café au lait spots are a type of birthmark characterized by flat patches on the skin. They are light brown in color but can darken with sun exposure. These marks are distinct because they often have irregular edges and vary in color.
When do you get spots on your skin?
Some spots are present at birth, whereas others start to develop before the age of five. Because this genetic condition can cause bone abnormalities and language difficulties, see a doctor if you (or your child) have colored patches on the skin, along with lumps under the skin or speech problems.
Can you remove a café au lait?
Café au lait spots are a benign and harmless condition. They don’t require treatment . One option to make these spots less noticeable is to get laser treatment. But even if you remove these spots, they could return later. You can also apply makeup to hide café au lait spots.
Do you need to treat a café au lait?
The outlook for café au lait spots is positive and these birthmarks don’t need treatment. But if you prefer having these spots removed, talk to your doctor about laser treatment or use make-up to hide discoloration. Last medically reviewed on January 22, 2018.
Can a café au lait rash cause cancer?
Developing a spot on your body can be a cause for concern, but café au lait spots are benign pigmented lesions that don’t cause cancer . These spots are usually smooth, although some spots can be raised.
What causes café-au-lait spots?
Cafe-au-lait spots can form when pigment-producing cells called melanocytes group together under the skin. Some babies have them at birth, but they can also develop during early infanthood. The spots can occur in all skin tones, though they seem to affect Black babies slightly more often.
What percentage of people have a café au lait spot?
Chances are, it’s a café-au-lait spot, a common type of birthmark. Café-au-lait spots are found in 20 percent to 30 percent of people, usually from birth. What’s more, they’re rarely cause for concern and aren’t usually treated. av-override. ‒‒:‒‒.
What do café-au-lait spots in babies look like?
Think a very large, pale version of a freckle. These common spots look a lot like their name suggests: Café-au-lait means “coffee with milk.”
Why are my kids' armpits jagged?
And children with more than five café-au-lait spots, especially spots in the armpits or groin, may be more likely to have neurofibromatosis. If your little one’s café-au-lait spot is bigger than her hand, is jagged instead of smooth or is part ...
Can babies have café au lait?
The spots can occur in all skin tones, though they seem to affect Black babies slightly more often. Most café-au-lait spots aren’t harmful and don’t turn into skin cancer.
Is it okay to have a café au lait spot on your baby?
It’s worth pointing out any kind of skin spot to your baby’ s pediatrician, including café-au-lait marks. While the vast majority are harmless, café-au-lait spots and other birthmarks are always worth keeping an eye on.
How big are 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
How many spots are there in a child with café au lait?
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.
What are the brown spots on my body?
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.
What does "cafe au lait" mean?
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.
Can you have multiple cafe au lait spots without Nf1?
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.
Is a café au lait spot a sign of 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).
Is a café au lait permanent?
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.
What causes café-au-lait macules?
The brown colour of a café-au-lait macule is due to a pigment called melanin, which is produced in the skin by cells called melanocytes .
What is a café au lait?
A café-au-lait macule is a common birthmark, presenting as a hyperpigmented skin patch with a sharp border and diameter of > 0.5 cm. It is also known as circumscribed café-au-lait hypermelanosis, von Recklinghausen spot, or abbreviated as 'CALM'. Café-au-lait macules.
When do café au lait macules appear?
Café-au-lait macules are usually present at birth ( congenital) or appear in early infancy. They sometimes become apparent later in infancy, especially after exposure to the sun, which darkens the colour.
Is a café au lait macules a melanocytic nae?
A café-au-lait macules is not classified as a congenital melanocytic naevus .
How many cafe au lait spots are there?
Signs and symptoms include: Flat, light brown spots on the skin (cafe au lait spots). These harmless spots are common in many people. Having more than six cafe au lait spots suggests NF1. They are usually present at birth or appear during the first years of life. After childhood, new spots stop appearing.
What is neurofibromatosis caused by?
Neurofibromatosis is caused by genetic defects (mutations) that either are passed on by a parent or occur spontaneously at conception. The specific genes involved depend on the type of neurofibromatosis:

What Do Café-Au-Lait Spots in Babies Look like?
What Causes Café-Au-Lait Spots?
- Cafe-au-lait spots can form when pigment-producing cells called melanocytes group together under the skin. Some babies have them at birth, but they can also develop during early infanthood. The spots can occur in all skin tones, though they seem to affect Black babies slightly more often. Most café-au-lait spots aren’t harmful and don’t turn into s...
Do Café-Au-Lait Spots Go away?
- Your little one will have her café-au-lait spot for life (unless you opt to have it removed with laser treatments, which is rarely done). And chances are, it will grow as she gets older and darken as she spends more time in the sun. Consider it just one more special thing that makes her unique.
When to Talk to Your Baby’S Doctor About Café-Au-Lait Spots
- It’s worth pointing out any kind of skin spot to your baby’s pediatrician, including café-au-lait marks. While the vast majority are harmless, café-au-lait spots and other birthmarks are always worth keeping an eye on. Rarely, café-au-lait spots can be linked to certain health conditions. Large marks with jagged edges are tied to McCune-Albright syndrome, a rare genetic condition. …
Overview
Café au lait spots, or café au lait macules, are flat, hyperpigmented birthmarks. The name café au lait is French for "coffee with milk" and refers to their light-brown color. Café au lait lesions with rough borders ("coast of Maine") may be seen in McCune-Albright syndrome. In contrast, Café au lait lesions of neurofibromatosis have smooth borders ("coast of California").
Prognosis
Café au lait spots are usually present at birth, permanent, and may grow in size or increase in number over time.
Cafe au lait spots are themselves benign and do not cause any illness or problems. However, they may be associated with syndromes such as Neurofibromatosis Type 1 and McCune-Albright syndrome.
Cause
Café au lait spots can arise from diverse and unrelated causes:
• Ataxia–telangiectasia
• Basal cell nevus syndrome
• Benign congenital skin lesion
• Bloom syndrome
Diagnosis
Diagnosis is visual with measurement of spot size. The number of spots can have clinical significance for diagnosis of associated disorders such as Neurofibromatosis type I. Six or more spots of at least 5mm in diameter in pre-pubertal children and at least 15mm in post-pubertal individuals is one of the major diagnostic criteria for NF1.
Treatment
Café au lait spots can be removed with lasers. Results are variable as the spots are often not completely removed or can come back after treatment. Often, a test spot is treated first to help predict the likelihood of treatment success.
See also
• Birthmark
• Nevus
• List of cutaneous conditions
• List of conditions associated with café au lait macules
External links
• eMedicine