Morphea of the face

What is morphea of the face?

Morphea is a type of localized scleroderma, which means “hard skin.” It happens when the body makes too much collagen, causing skin to thicken and tighten. Unlike systemic scleroderma, morphea does not affect the internal organs.

When morphea appears on the face, it can cause cosmetic and functional changes. While it may sound worrying, most children respond well to treatment and live active lives.

Types of morphea that affect the face

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

• En coup de sabre (the strike of the sword) appears on the forehead and sometimes the scalp as a linear streak or sunken skin that can be discoloured.
• Parry-Romberg syndrome: Affects one side of the face, usually below the forehead, involving both skin and deeper tissues. This can lead to changes in skin colour, shrinkage of tissue and differences in facial size or shape, especially as a child grows.

Morphea looks like discoloured patches on the skin

• Discoloured patches or streaks on the face, often starting red-pink then becoming whitish with a purple edge.
• The skin may become shiny, firm or feel harder than normal.
• Veins may show more clearly, and the skin may look thinner, raised, or sunken compared to surrounding areas.
• Hair loss can occur if the scalp is affected.

Because morphea looks different from child to child, it can sometimes be mistaken for bruises or other conditions.

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 and cannot be passed on to others.

How long does morphea of the face last?

Morphea of the face is unpredictable. It may last for months or years before it is noticed. Even without treatment, the active inflammation usually settles after three to five years.

There are typically three phases:

1. Early phase: redness and inflammation. 
2. Active phase: pale or whitish centre with a purple-red border, skin hardening.
3. Inactive (burnt-out) phase: the disease quiets down, but the skin may remain thin, darker or lighter in colour, or sunken (due to loss of fat).

Some children continue to have disease activity for longer and may need ongoing treatment.

Morphea is diagnosed by history and examination

Your child’s health-care provider can diagnose morphea by asking about your child’s medical history and examining your child’s skin. A skin biopsy (small skin sample) may sometimes be taken. There is no blood test that can confirm morphea, but other tests such as an ultrasound or MRI may be used to check for changes in deeper tissues, the eyes or brain if needed.

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, mycophenolate and/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 child’s health-care provider 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.

An 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.

Preventing complications from morphea of the face

You cannot prevent morphea from developing, but early diagnosis and treatment reduce the chance of complications. Follow the health-care team’s recommendations for screening of associated complications (eye, brain, dental and/or psychological).

Skin changes

When the disease becomes inactive, the affected skin may become darker (or sometimes lighter) than the rest of the skin. Additionally, hardening of the skin may leave permanent scarring. Loss of fat may make the skin look 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 can be permanent.

Associated brain changes

Some children may experience headaches. Rarely, complications such as seizures and brain inflammation may happen. Your child’s health-care provider may ask for an MRI scan of the brain to see if there are any changes. 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. Their health-care provider will recommend an eye exam to check for inflammation.

Associated mouth and dental changes

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

How to help your child with morphea of the face

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. 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.

When to seek medical attention

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

If your child has seizures or becomes weak, take them to the nearest Emergency Department and notify their health-care team.

Resources for families

National Scleroderma Foundation: https://scleroderma.org/

Scleroderma Canada: https://www.scleroderma.ca/

JAMA Dermatology Patient Page – Morphea (Localized Scleroderma): https://jamanetwork.com/journals/jamadermatology/fullarticle/1740691

References

Fett NM. (2013). Morphea (localized scleroderma). JAMA Dermatology, 149(9):1124. doi:10.1001/jamadermatol.2013.5079

Penmetsa GK, Sapra A. (2023). Morphea. In StatPearls. StatPearls Publishing. Retrieved from: https://www.ncbi.nlm.nih.gov/books/NBK559010/

Zulian F. (2017). Scleroderma in children. Best practice & research. Clinical rheumatology, 31(4):576-595. doi: 10.1016/j.berh.2018.02.004.