What Is Nevus Sebaceous Syndrome?

Medically Reviewed by Melinda Ratini, DO, MS on June 03, 2021

Nevus sebaceous syndrome is characterized as a disorder that displays as a skin lesion in the sebaceous glands of your skin. The condition causes the production of too many sebaceous glands along with an overgrowth of skin in the same area.

Understanding Nevus Sebaceous Syndrome

Nevus sebaceous syndrome encompasses a series of unusual health conditions that appear together on your skin and in your body. This syndrome is also called:

  • Schimmelpenning syndrome
  • Jadassohn nevus phacomatosis
  • Jadassohn sebaceous nevus syndrome
  • linear sebaceous nevus sequence
  • Schimmelpenning-Feuerstein-Mims syndrome‌
  • Epidermal nevus syndrome

A nevus sebaceous is a mole patch that is salmon or yellow-colored and most often appears on your scalp, neck, and face. They are less common on your arms, legs, and trunk. Sebaceous nevi usually appear as salmon or yellowed-colored, hairless, and smooth patches. They tend to develop in the womb and may or may not be obvious at birth.

If they appear on your scalp, the area won’t have any hair. This is often called alopecia. Aside from the way they look, they don’t usually cause any negative symptoms. During puberty, they may change with adjusting hormone levels to become more obvious. The texture is often thick, scaly, and wart-like.

These spots tend to appear alone. They become part of a syndrome when there are other accompanying health conditions like:

  • Seizures
  • Delays in reaching developmental milestones
  • Intellectual impairment
  • Damage to some cranial nerves‌
  • Abnormalities in some brain structures

Brain development. Abnormalities of the brain include one side of your brain becoming enlarged. This leads to brain vessels not forming correctly, nerves that connect your two brain hemispheres missing, small or missing folds in your brain that are traditionally more prominent, or folds that are thicker than they should be.

Other syndromes. Dandy-Walker malformation is also common with nevus sebaceous syndrome. This congenital birth defect leaves an unusually large space at the back of your brain. It interferes with the way cerebrospinal fluid flows, causing fluid buildup and pressure around your brain. This causes neurological impairment, including delays in motor skill development and difficulty learning.

Ocular concerns. Nevus sebaceous syndrome often includes eye issues. You may be missing part of the ocular tissue from your eye coloring or the back of your eye, also called the retina. Your eyes may appear cloudy or they may cross. If your optic nerve is damaged as part of the syndrome, it may impact your ability to see. Damage may be caused by scarring, nerve damage, or a tumor.

Bone concerns. Skeletal malformations are also common and may include:

  • Significant curving of your spine
  • Frequently dislocated hips
  • Limb deformities
  • Bone cysts
  • Prominent forehead, asymmetrical skull, and other craniofacial defects ‌
  • Additional bony structures around ankle and foot joints

Because it is common to be resistant to vitamin D, your bones may bow out as your bones soften over time. Children may not grow as fast as their peers and may be shorter as adults and prone to fractures.

What Causes Nevus Sebaceous Syndrome?

Research suggests that after conception, two genes called KRAS and HRAS mutate to cause nevus sebaceous syndrome. Men and women are both affected equally by the condition. The skin lesions with or without the syndrome are present in about one to three babies per 1,000 live births.

Diagnosing Nevus Sebaceous Syndrome

After birth, your pediatrician assesses your baby’s overall health. If there is an obvious mole, they may look for other signs of nevus sebaceous syndrome. A diagnosis is made based on other characteristic symptoms. If your doctor wants to confirm their suspicions, they may take a small tissue sample from the nevus sebaceous for further testing.

Additionally, your baby may need an x-ray or MRI to assess the bone structure and look for deformities. If your doctor thinks the risk of imaging outweighs the benefits, they may want to wait until your infant is older. In this case, the doctor monitors your baby’s development closely to watch for milestones and signs that your baby needs treatment for the condition.

Treating Nevus Sebaceous Syndrome

When your child is young, your doctor may want to watch and wait unless there are obvious deformities that need to be addressed. If the mole itself grows too much, your doctor may suggest removing it before puberty. Even without causing additional health problems, it may lead to feeling self-conscious about its appearance. Surgery at a younger age may be easier to recover from than waiting until later.

Your doctor may refer your child to a specialist for other concerns. These doctors may include:

  • Pediatricians
  • Pediatric neurologists
  • Dermatologists
  • Orthopedists
  • Orthopedic surgeons‌
  • Ophthalmologists

Together, this team of professionals works to address your child’s needs and improve their quality of life. Your child may need any combination of medication, procedures, and therapy. A lot of decisions are based on your child’s overall health and tolerance for treatment.

The doctors will talk to you about the benefits and risks so you can decide which treatment options you want to pursue. In some cases, treatments may be better suited later in life when your child is older.

WebMD Medical Reference



Nationwide Children’s: “Nevus Sebaceous.”

Rare Diseases: “Nevus Sebaceous Syndrome.”

UpToDate: “Nevus sebaceous and nevus sebaceous syndrome.”

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