Morphea

What is morphea?

Morphea is a skin condition 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. 

There are several subtypes of morphea, depending on the appearance and extent of the skin lesions. The separation helps to decide on the proper management.

Plaque morphea

Morphea looks like discoloured patches on the skin

Morphea appears as patches called "plaques" or "lesions" on the skin. It usually begins with a whitish patch with a purple border. It often starts with the appearance of a bruise. However, it can look very different from person to person. There are several types of morphea, and each type looks a little different:

• Plaque morphea usually begins with a reddish patch that, over time, becomes an oval shape with a yellow or ivory colour in the centre and purple at the edges. It may feel thick, firm and waxy, and may look shiny. It often has the appearance of a bruise. 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.
  
• Generalized morphea occurs when many plaques are noted or more than one body part is affected.
  
• Linear morphea looks similar to plaque morphea, except it tends to follow a line along an arm or leg.
• Pansclerotic morphea is a very rare variant of morphea where most of the skin becomes hard and stiff.

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 is rare and there is no known cause

Sometimes morphea seems to happen after an injury. Morphea may be the result of an overactive immune system (autoimmune disease). Morphea is not contagious.

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

The disease has three phases:

• an early inflammatory phase that presents as nonspecific redness
• a second inflammatory 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)

Some patients may have disease activity beyond the three to five years that requires longer-term treatment.

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 for 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:

• 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.

Ultrasound or MRI may be used to see if the morphea has affected the deeper tissues under the skin.

Physiotherapy

Children with limitation in movement may benefit from therapy to increase the function of the joint.

Surgery

Some children with morphea may be candidates for orthopaedic or plastic surgery to deal with scarring or deformities 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.

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.

Musculoskeletal involvement

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

Deformity

The affected area may 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.

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 and 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 inflammation of morphea may last three to five years, even if not treated. Most children recover very well.

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.

Permanent scarring or differences in the appearance or size/length of the extremity may occur.

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.