Morphea

What is morphea?

Morphea is a skin condition known as localized scleroderma, meaning "hard skin." The skin becomes thick, tight and hard because it contains too much collagen and other proteins. There may be changes in skin colour and texture.

Unlike systemic scleroderma, morphea does not affect internal organs. Morphea affects only the skin and sometimes the tissues underneath. 

There are different subtypes of morphea, depending on how the patches look and where they appear. Most children improve with treatment and can lead active lives.

Plaque morphea

Morphea looks like discoloured patches on the skin 

Morphea usually starts as patches of discoloured skin. At first, these patches may look like bruises or pale white areas with a purple edge. Over time, they can become harder, shiny, or feel thicker than the surrounding skin. The skin may look raised or sunken, and veins underneath can sometimes show more clearly.

There are different types of morphea, which can look a little different: 

• Plaque morphea: Oval or round patches that begin reddish, then turn ivory or yellow in the centre with a purple border. It often looks like a bruise. The skin may feel firm, waxy or shiny.
• Generalized morphea: Many plaques appear, or more than one body area is affected.
• Linear morphea: A streak or line of hardened skin, usually on an arm, leg or sometimes the face.
• Pansclerotic morphea: A very rare variant of morphea where large areas of the body, or most of the skin, become stiff and hard.

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. Because morphea can look quite different from person to person, it may be difficult to recognize at first.

Morphea is rare, and there is 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 last?

Morphea is unpredictable. Most children have the disease for months or even years before the condition is recognized or diagnosed. Even without treatment, morphea often becomes inactive or "burnt out" after approximately three to five years, though the skin changes may remain.

The disease has three phases:

• Early phase: The skin looks red or inflamed.
• Active phase: The middle of the patch becomes pale or white, with a red-purple border. The skin feels harder at this stage.
• Inactive (burnt-out) phase: The patch stops spreading, but the skin may look darker or lighter, thinner or sunken (due to loss of fat), with veins showing more clearly.

Some children may have active disease for longer than five years and may need longer-term 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.

They might also take a small part of the skin to send to the lab for tests. This is called a skin biopsy. There is no blood test to confirm the diagnosis for morphea, although the health-care providers will ask for tests to check how other organs are functioning.

There are several treatments for morphea

Morphea can be controlled, but it cannot be cured. Treatment aims to 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:

• is spreading rapidly
• affects a younger child
• affects large areas

Your child may need regular blood tests to monitor the effects and possible side effects of the medication.

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.

Physiotherapy

Children with limited movement may benefit from therapy to increase the function of the affected joint.

Surgery

Some children with morphea may be candidates for orthopaedic or plastic surgery to deal with scarring or joint problems and can be referred for a consultation.

Preventing complications of morphea

You cannot prevent morphea, but starting treatment early reduces the chance of complications. Follow the health-care team’s recommendations for screening of associated complications.

Skin changes

When the disease becomes inactive, the affected skin may become darker 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.

Movement problems

Some children may have arthritis or develop limitation in the movement of a joint. Rarely, deeper involvement of the muscle and fascia (connective tissue) may occur. 

Growth differences

The affected area may look smaller or grow more slowly. 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.

How to help your child with morphea

Discuss the diagnosis openly with your child and provide reassurance.

Discuss the diagnosis with teachers, especially if your child will 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.

Encourage your child to be active.

Outcome

The active phase of morphea usually lasts about three to five years, even without treatment. Most children do very well, and the condition eventually settles down.

After the patches fade, the skin may still look a bit different. It might be darker or lighter in colour or feel thinner than the surrounding skin. Sometimes the fatty layer under the skin shrinks, which can make the area look sunken or have a different texture.

In some cases, there may be permanent scarring or differences in the size or shape of a limb, especially if morphea developed during a child’s growth years.

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.

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