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Cutis aplasia

What is cutis aplasia?

Cutis aplasia (KYOO-tiss uh-PLAY-zhuh), also called aplasia cutis congenita ( kohn-JEN-uh-tuh) (ACC), is a rare condition a baby is born with where skin is missing in one or more places. If affects around one to three out of 10,000 births.

Although it can happen anywhere on the body, commonly it's a single place on the scalp at the very top of the head. Usually, the spot is small, no more than 1 to 2 centimeters across, which is about the width of a penny. The depth of the spot can vary and no two spots are alike.

In about 15 to 30% of these places, called lesions, the bone and other tissues underneath are also affected. When these lesions are on the scalp, hair is usually missing there as well.

Lesions usually have a definite border. Their appearance can fall generally into one of two categories:

• Membranous: These types of lesions are usually round or oval and may stick out. They are typically covered by transparent, shiny tissue and may be filled with fluid. Sometimes it’s possible to see through the lesion to the tissue and bone underneath. They may be part of an underlying abnormality of the nervous system. Membranous lesions are more common than nonmembranous.
• Nonmembranous: These types of lesions don’t have a circular shape. They can be rectangular or star shaped. They usually look like an ulcer or an open wound because they do not have the shiny transparent tissue over the top. Sometimes, they have healed before birth and look like a scar.

In about 15% of cases, ACC lesions occur on the trunk, arms and/or legs. These places are usually larger than lesions on the scalp, but they also usually heal faster. These commonly look like wounds or ulcers.

What causes cutis aplasia?

Experts don’t completely understand what causes ACC, but in general it seems to be a problem that prevents the skin from developing normally in the womb, or that destroys skin before the baby is born. Most likely, more than one thing can cause it.

A few possible causes are:

• Infections in the womb
• Genetic abnormalities
• Use of certain medications during pregnancy
• Problems with blood flow in the womb
• Injury to the baby before birth
• Problems in the baby’s brain, spine or spinal cord
• A hole the in membrane around the womb during pregnancy

Most of the time, ACC lesions happen without any other problems anywhere else. Rarely, they are part of a syndrome that can cause problems in other places in the body. Some syndromes that can cause ACC are Adams-Oliver, Bart, Johanson-Blizzard and Setleis syndromes.

How is cutis aplasia diagnosed?

Usually, lesions from ACC are found at birth. If the lesion has no other symptoms, which would be typical for just one small place on the head, doctors may not need to do more testing.

However, for larger lesions that are open wounds, or ones of any size that have features that sometimes signal other concerns, doctors might perform tests, such as an MRI, to make sure there aren’t underlying problems that need treatment. They will likely do a thorough physical exam to check for problems anywhere else on the body.

ACC isn’t the only type of skin problem that can show up at birth, so doctors have to rule out other causes. Other types of skin problems that might look like ACC include:

• Meningocele (men-IN-go-seel), a sac sticking out from the head that contains tissue from the brain and/or spine
• Congenital dermoid cysts
• Injuries that happened during delivery
• Sores from electrodes placed on the baby’s head for monitoring
• Epidermolysis bullosa (ep-ih-dur-MOL-uh-sis buhl-LOE-sah), an inherited disease that causes extremely fragile skin
• Infections
• Transient bullous dermolysis (TRAN-zee-ent BOOL-us dur-MAH-li-sis), a rare condition that causes blistering in newborns. This condition goes away on its own.

How is cutis aplasia treated?

Treatment for an ACC lesion will depend on how big and deep it is, and where it is located.

Fortunately, most lesions are just a single, small spot on the scalp that are not very deep. These will likely go away on their own over a few weeks to two months, and leave a scar that may not grow hair. The treatment for these lesions, generally smaller than 4 centimeters (about 1.5 inches), is to prevent infections by keeping them clean and using antibacterial ointment.

About 10 to 30% of the time, small scalp lesions may be associated with other problems. Doctors may perform more tests, such as imaging or blood work, if the lesion has traits that sometimes point to other concerns. Some things doctors watch include a reddish color, signs of infection or a ring of long dark hair around the lesion (a “hair collar”).

For a lesion on the scalp that is larger than four centimeters, doctors may perform imaging tests such as an ultrasound or MRI. These tests help them to see exactly how deep the lesion goes and if bone or tissues underneath are affected. Larger lesions have a greater risk of bleeding and infection, which can turn serious. In some cases, there may be a need for surgery to close the opening. In others, careful monitoring with good attention to keeping the area clean, moist and protected from infection may be enough.

Large lesions that are not on the baby’s head can usually be treated without surgery. They will typically heal on their own by keeping the area clean, moist and protected from infection.

Every case is different, so it’s important to get care from a medical team that includes all the experts that babies may need for the right treatment. This could include:

• Pediatrician
• Pediatric dermatologist
• Pediatric plastic surgeon
• Pediatric neurosurgeon

What is the outlook for cutis aplasia?

Small single lesions on the scalp without any underlying concerns typically don’t cause problems. They usually go away on their own in about a month. There will usually be a scar that won’t grow hair. The scar can be removed surgically, once the child is old enough, if there is concern about how it looks.

Lesions on the scalp that are larger or associated with other problems may require more focused treatment to prevent bleeding, blood clots, infections and other possible serious complications. Also, if a child has a lesion that is part of a syndrome, all the possible symptoms of the syndrome will need to be addressed. In addition to medical treatment, these families may be referred for genetic counseling.

Get help at Children’s of Mississippi

If your child has cutis aplasia, you can schedule an appointment with our Children’s of Mississippi plastic surgery team. They are experts in evaluating and treating lesions anywhere on the body. They can answer your questions and help you understand the options for your child. Schedule an appointment online.

Last reviewed: 8/4/2026