Morphea of the face

What is morphea of the face?

Morphea is a skin condition also known as localized scleroderma, meaning "hard skin." The skin becomes thick and hard because it contains too much collagen and other proteins.

There are two types of scleroderma: a systemic type and a localized type. Localized scleroderma must not be confused with systemic scleroderma. The localized type affects the skin only and does not affect the internal organs. 

When this condition appears on the face, it is called "morphea of the face."

Morphea of the face

There are two types of morphea that can affect the face:

• En coup de sabre (hit by the sabre) appears on the forehead and sometimes the scalp as a linear streak or sunken skin with pigmentation changes.
• Parry-Romberg Syndrome appears on the face below the forehead. This type of morphea affects the skin as well as the tissues under it. The colour of the skin might change, and the tissues underneath may appear sunken or smaller. If the morphea affects one side of the face, this side might look smaller than the other side. As the child grows, the difference in the size and shape of that part of the skin may become more obvious, even if there is no ongoing inflammation of the skin.

Morphea looks like discoloured patches on the skin

Morphea makes patches called "plaques" or "lesions" on the skin. It usually begins with a reddish patch that, over time, becomes waxy white with a purple border. It often has the appearance of a bruise. However, it can look very different from person to person.

As morphea progresses, the affected skin may change. The veins under the skin may show more clearly. The affected skin may look thinner, and may look raised or sunken compared to the skin around it.

Morphea has no known cause

Sometimes morphea seems to happen after an injury. Morphea likely results from an overactive immune system (autoimmune disease). Morphea is not contagious.

The disease is unpredictable. Most children have the disease for months and even years before it becomes recognized or diagnosed. Even without treatment, morphea stops being active and it usually “burns out” after three to five years.

The disease has three phases:

• an early inflammatory phase that presents as nonspecific redness
• an ongoing active phase, which typically presents with a hardened middle of white discolouration with a red-purple border
  
• a burnt-out phase, appearing as increased colour, thinning of the skin with more visible vessels or sunken skin layers (due to loss of fat)

Morphea is diagnosed by history and examination

The doctor might take a small part of the skin to send to the lab for tests. This is called a biopsy. There is no blood test to confirm the diagnosis of morphea, although the doctors will ask for tests to look for functioning of other organs.

There are several treatments for morphea

Morphea can be treated, but it cannot be cured. Treatment can reduce the inflammation, stop lesions from spreading and prevent new lesions from appearing.

Creams, ointments and medication

You may be given ointments or creams to put on your child's skin.

Your child may need stronger medication, such as prednisone or methotrexate, if the morphea:  

• starts at a younger age
• affects large areas
• is spreading rapidly

Your child may need to have regular blood tests in these cases. These tests monitor the effects and possible side effects of the medication. Occasionally, other medicine may be needed as well.

Cosmetics

There are special make-up products that can cover the darker or lighter parts of the skin. Ask your doctor about these products.

Monitoring

At each clinic visit, your child's skin will be examined. Photographs will be taken to help keep track of changes in the morphea.

Ultrasound or MRI may be used to see if the morphea has affected the deeper tissues under the skin and to look for associated changes in the brain.

Cosmetic surgery

Some children with morphea of the face may be candidates for plastic surgery and can be referred for a consultation.

Most of the complications related to the disease can be decreased by treating it early. Follow the health-care team’s recommendations for screening of associated complications (eye, brain, psychological).

Skin changes

When the disease becomes inactive, the affected skin may become darker (or occasionally lighter) than the rest of the skin. Additionally, hardening of the skin may leave permanent scarring. Loss of fat may make the skin appear thinner with more visible vessels.

Cosmetic impact

Morphea may cause the affected side of the face to look smaller. The difference in size compared with the normal side may become more obvious when the child grows, even if the disease is no longer active.

Hair loss

If the morphea affects the scalp, it may lead to hair loss that is permanent in most cases.

Associated brain changes

Some children may experience headaches. Rarely, complications such as seizures and brain inflammation may happen. Your doctor may ask for an MRI of the brain to see if any changes are present. Some changes, such as calcium deposits, do not have any associated symptoms.

Associated eye changes

Some children may have inflammation of the eye, even if they do not have any symptoms. The doctor will recommend an eye exam.

Associated mouth and dental changes

In some children, especially with Parry-Romberg Syndrome, differences in the size and growth of the mouth may occur. These children may require orthodontic treatment.

Discuss the diagnosis openly with your child and provide reassurance.

Discuss the diagnosis with teachers, especially if your child would require multiple hospital visits and treatments and to prevent bullying.

Seek reassurance and coping strategies from your child’s health-care team and ask for a referral to a mental health specialist if your child is becoming anxious or depressed or is facing bullying.

Outcome

The active inflammation phase of morphea of the face usually lasts about three to five years. The majority of children recover well from morphea of the face.

After the morphea is gone, there may be some change in the pigment (colour) of the skin.

The skin and other tissues may atrophy (shrink). The fatty tissue under the skin may disappear. This will make affected areas look thinner and have a different texture.

If your child is on methotrexate or prednisone and becomes unwell, has fever or has been exposed to chickenpox and is not vaccinated, stop the medication and contact your child’s health-care team for further information.

If your child has seizures or become weak, take them to the nearest emergency department and notify their health-care team.