What causes café-au-lait spots in adults?

What causes café-au-lait spots? An increase in the number of skin cells (melanocytes) that produce the protective pigment that darkens skin (melanin) in the thin outer layer of your skin (epidermis) causes café-au-lait (CAL) spots.
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What can cause café-au-lait spots?

Café au lait spots can arise from diverse and unrelated causes:
  • Ataxia–telangiectasia.
  • Basal cell nevus syndrome.
  • Benign congenital skin lesion.
  • Bloom syndrome.
  • Chédiak–Higashi syndrome.
  • Congenital melanocytic naevus.
  • Fanconi anemia.
  • Gaucher disease.
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Should I worry about café-au-lait spots?

Café au lait spots are usually harmless and don't cause any uncomfortable symptoms or complications. But you shouldn't ignore these spots, especially if you have more than a handful on your body. This could indicate an underlying genetic disorder.
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When are café-au-lait spots significance?

The spots are usually present at birth but may develop later in life. Café au lait spots are harmless and normal, with some people having anywhere from one to three spots. But sometimes, these spots can indicate an underlying genetic problem.
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Can you have café-au-lait spots without neurofibromatosis?

It is not unusual for a healthy child to have a few café-au-lait spots, and it is not recommended to provide an evaluation for NF1 if the spots are fewer than six in number. These spots also should be clear and distinct – finding very faint or small skin spots does not count towards the six spots needed for diagnosis.
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Café-au-lait spots



Do café-au-lait spots run in family?

Abstract. 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.
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Can multiple café-au-lait spots be normal?

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.
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At what age is neurofibromatosis usually diagnosed?

Neurofibromatosis 1. 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.
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Does neurofibromatosis worsen with age?

Unfortunately, NF1 can worsen with time, resulting in new growths over the body that cause major psychological and cosmetic issues.
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Can café-au-lait spots appear later in life?

Can café-au-lait spots suddenly appear in adulthood? Yes. Café-au-lait (CAL) spots are most often present on a newborn's skin but it is common for CAL spots to develop on a person's skin later in life. It is normal for a person to have a few CAL spots but more than six spots may be a sign of an underlying condition.
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How is neurofibromatosis treated in adults?

There is no known treatment or cure for neurofibromatosis or schwannomatosis. Medication can be prescribed to help with pain. In some cases, growths may be removed surgically or reduced with radiation therapy.
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Can adults be diagnosed with neurofibromatosis?

The disorder is usually diagnosed in early childhood since some of the symptoms are present in infants or even newborns. In some cases however, the symptoms appear in adulthood only when they might cause diagnostic problems.
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What can be mistaken for neurofibromatosis?

Legius syndrome also is called a neurofibromatosis 1-like syndrome because its symptoms are similar to NF1. Individuals with Legius syndrome have skin problems including brown birthmarks, called cafe-au-lait spots, and freckling, as well as mild learning problems and a larger head.
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What is the life expectancy of a person with neurofibromatosis?

If there are no complications, the life expectancy of people with NF is almost normal. With the right education, people with NF can live a normal life. Although mental impairment is generally mild, NF1 is a known cause of attention deficit hyperactivity disorder.
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Who is most likely to get neurofibromatosis?

The only true risk factor for developing neurofibromatosis is having a parent who also has the condition. In nearly half of all cases of type 1 and type 2 neurofibromatosis, and in around 15 percent of schwannomatosis cases, the condition is passed down from parent to child.
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How many café-au-lait spots are concerning?

Cafe au lait spots usually appear singly. More spots can occur in children with a genetic condition called neurofibromatosis or other conditions. If your child has six or more cafe au lait spots, ask your doctor whether your child needs a medical examination.
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What tests are done to diagnose neurofibromatosis?

Imaging tests.

X-rays, CT scans or MRIs can help identify bone abnormalities, tumors in the brain or spinal cord, and very small tumors. An MRI might be used to diagnose optic gliomas. Imaging tests are also often used to monitor NF2 and schwannomatosis.
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Is NF1 serious?

The symptoms of neurofibromatosis type 1 (NF1) are often mild and cause no serious health problems. But some people will have severe symptoms. The symptoms of NF1 can affect many different areas of the body, but it's unlikely someone will develop all of them.
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Are brain tumors common in adults with NF1?

The most common brain tumor affecting individuals with NF1 is the OPG, seen in 15% to 20% of people with this condition. These neoplasms are classified as pilocytic astrocytomas, which do not progress to high-grade malignancies.
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What do neurofibromas look like at first?

Typically, people with NF1 start to see neurofibromas appear during their teens. The neurofibromas grow slowly and may look like a pimple at first. You won't wake up one morning, or next year, and be covered with neurofibromas. They develop gradually over a period of many years.
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Is neurofibromatosis an autoimmune disease?

Discussion. Neurofibromatosis type 1 is a relatively common autosomal dominant disorder. It is well known that NF1 patients have an increased risk of developing a variety of benign and malignant tumors, but its association with autoimmune diseases has been rarely reported (1-3).
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What part of the body does neurofibromatosis affect?

Neurofibromatosis (NF) is a genetic neurological disorder that can affect the brain, spinal cord, nerves and skin. Tumors, or neurofibromas, grow along the body's nerves or on or underneath the skin.
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Does diet affect neurofibromatosis?

Excessive consumption of saturated fatty acids and lipids was also observed in both male and female patients. Conclusions: In this study, NF1 patients consumed an unhealthy diet that was rich in fats and sodium and lacking in fiber, vitamins, and minerals.
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What are the 3 types of neurofibromatosis?

There are three main types of neurofibromas: cutaneous, subcutaneous, and plexiform.
  • Cutaneous Neurofibromas. ...
  • Subcutaneous Neurofibromas. ...
  • Plexiform Neurofibromas. ...
  • Tumors of the Eyes. ...
  • Bone Abnormalities. ...
  • Hypertension. ...
  • Learning Problems.
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What is the difference between neurofibroma and neurofibromatosis?

Most neurofibromas occur in association with a genetic disorder called neurofibromatosis type 1 (NF1). This condition can lead to multiple neurofibromas and other symptoms. A person with NF might have a few neurofibromas, or hundreds. Solitary neurofibromas can also occur in people who don't have NF.
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