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Scleroderma in Children

What is scleroderma?

Scleroderma is an ongoing (chronic) disease that causes abnormal growth of connective tissue. It can affect the joints, skin, and internal organs. It is a rare degenerative disease and gets worse over time.

There are 2 main types of scleroderma: localized and systemic.

Localized scleroderma. The disease only affects 1 area of the body. This form of the disease is seen more often in children. It may involve patches of skin on the trunk, arms, legs, or head.

Systemic scleroderma. This type affects the whole body. It rarely occurs in children.

What causes scleroderma?

Scleroderma is thought to be an autoimmune disease. This means the symptoms are caused by the body attacking its own healthy tissues. Genes play a role in the disease, but it is not passed on from parents to children. Some environmental factors may also play a role.

What are the symptoms of scleroderma?

Scleroderma can lead to scarring of the skin, joints, and internal organs. Symptoms can occur a bit differently in each child, and may include:

  • Thick, swollen fingertips
  • Pale and tingly fingers that may turn numb when out in the cold or during emotional upset (Raynaud's phenomenon)
  • Joint pain
  • Taut, shiny, darker skin on large areas, that may cause problems with movement
  • Spider veins
  • Calcium bumps on the fingers or other bony areas
  • Frozen (immobile) fingers, wrists, or elbows due to scarring of the skin
  • Sores on fingertips and knuckles
  • Grating noise as inflamed tissues move
  • Scarring of the esophagus, leading to heartburn and trouble swallowing
  • Scarring of the lungs, leading to shortness of breath
  • Heart failure and abnormal heart rhythms
  • Kidney disease

Symptoms of localized sclerosis may include:

  • Shiny, thickened patches of skin
  • Lighter or darker (discolored) skin
  • Joint tightness

These symptoms can seem like other health conditions. Make sure your child sees his or her healthcare provider for a diagnosis.

How is scleroderma diagnosed?

Your child’s healthcare provider will take your child’s medical history and do a physical exam. Diagnosis is based on the changes in the skin and internal organs. An antibody test may help show the type of scleroderma. Other tests may also be done, such as:

  • Blood and urine tests. These can help find any problems with blood counts, or kidney or liver function.
  • Electrocardiogram (ECG). This test records the heart’s electrical activity, shows abnormal rhythms, and finds heart muscle damage. An ECG may be done to find changes in the heart muscle tissue due to scleroderma.
  • Echocardiogram. This test uses sound waves to create a moving image of the heart and its valves. It looks at the structure and function of the heart.
  • X-ray. This test uses a small amount of radiation to create images of internal tissues, bones, and organs. X-rays may show changes in bone, soft tissues, and organs caused by scleroderma.

How is scleroderma treated?

Your child's healthcare provider will figure out the best treatment plan for your child based on:

  • How old your child is
  • Your child’s overall health and medical history
  • How sick your child is
  • How well your child handles certain medicines, treatments, or therapies
  • If your child’s condition is expected to get worse
  • The opinion of the healthcare providers involved in your child's care
  • Your opinion and preference

Treatment may include:

  • Medicine to ease pain (nonsteroidal anti-inflammatory medicines or corticosteroids)
  • Medicine to slow the skin thickening process and delay damage to internal organs (penicillamine)
  • Medicine to weaken the immune system (immunosuppressive medicines)
  • Treatment of specific symptoms, such as heartburn and Raynaud's phenomenon
  • Physical therapy and exercise, to maintain muscle strength

Talk with your child’s provider about the risks, benefits, and possible side effects of all medicines.

What are the complications of scleroderma?

Complications of scleroderma vary depending on the type of the disease and how severe it is. Treatment may prevent or reduce problems. Possible complications may include:

  • Thickened, tight skin
  • Arthritis
  • Seizures
  • Behavior and learning problems
  • Vision changes
  • GERD (gastroesophageal reflux)
  • Less lung function
  • Heart and kidney damage

Helping your child live with scleroderma

Work with your child's healthcare provider. Together you can make a treatment plan. The plan can help your child be active as much as possible in school, and in social and physical activities. Make life as normal as possible for your child. Encourage exercise and physical therapy and find ways to make it fun. You can also help your child find a support group to be around other children with scleroderma. Work with your child's school to make sure your child has help as needed. Your child may also qualify for special help under Section 504 of the Rehabilitation Act of 1973.

When should I call my child’s healthcare provider?

Tell the healthcare provider if your child's symptoms get worse or there are new symptoms.

Key points about scleroderma

  • Scleroderma causes abnormal growth of connective tissue. It can affect the joints, skin, and internal organs.
  • Scleroderma can affect one area of the body, or affect the whole body. It is less common in children for it to affect the whole body.
  • Localized scleroderma may affect patches of the skin on the torso, arms, legs, or head.
  • There is no cure for scleroderma. Treatment is focused on relieving pain and slowing down damage to the body.

Next steps

Tips to help you get the most from a visit to your child’s healthcare provider:

  • Know the reason for the visit and what you want to happen.
  • Before your visit, write down questions you want answered.
  • At the visit, write down the name of a new diagnosis, and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you for your child.
  • Know why a new medicine or treatment is prescribed and how it will help your child. Also know what the side effects are.
  • Ask if your child’s condition can be treated in other ways.
  • Know why a test or procedure is recommended and what the results could mean.
  • Know what to expect if your child does not take the medicine or have the test or procedure.
  • If your child has a follow-up appointment, write down the date, time, and purpose for that visit.
  • Know how you can contact your child’s provider after office hours. This is important if your child becomes ill and you have questions or need advice.