When Your Child Has Scleroderma 

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

There are 2 main types:

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

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

How to say it

sklayr-oh-DER-muh

What causes scleroderma?

Healthcare providers think that scleroderma is 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.

Symptoms of scleroderma

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

Symptoms of localized sclerosis may include:

• Shiny, thickened patches of skin

• Lighter or darker (discolored) skin

• Joint tightness 

Symptoms of systemic sclerosis 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 because of scarring of the skin

• Sores on fingertips and knuckles

• Grating noise as inflamed tissues move

• Scarring of the esophagus, leading to heartburn and trouble or pain swallowing

• Scarring of the lungs, leading to shortness of breath

• Heart failure and abnormal heart rhythms

• Kidney disease

Diagnosing scleroderma

Your child’s healthcare provider will ask about your child’s health history. They will also 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. Your child may need other tests, 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. It shows abnormal rhythms and finds heart muscle damage. An ECG may be done to find changes in the heart muscle tissue.

• Echocardiogram. This test uses sound waves to make 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 make images of internal tissues, bones, and organs. X-rays may show changes in bone, soft tissues, and organs.

• Other tests. If your child has symptoms that suggest other organs may be affected, your child may need other tests. For instance, a chest X-ray, CT scan, or pulmonary function tests can tell if the lungs are involved. Esophageal motility studies can see if your child has a problem in their gastrointestinal (GI) tract.

Treatment for scleroderma

Treatment will depend on your child’s symptoms, age, and general health. It will also depend on how severe the condition is. Treatment may include:

• Medicine to ease pain, such as nonsteroidal anti-inflammatory drugs (NSAIDs) or corticosteroids

• Medicine called penicillamine to slow the skin thickening and delay damage to internal organs

• Medicine to weaken the immune system, called immunosuppressive medicine

• Phototherapy

• Treatment of symptoms, such as heartburn and Raynaud's phenomenon

• Physical therapy and exercise to keep up muscle strength 

• Surgery (often used only when a child stops growing)

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

Possible complications of scleroderma

Complications can vary. They depend on what type of scleroderma it is 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 disease) or heartburn

• Decreased lung function

• Heart and kidney damage

Living 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, social, and physical activities. Make life as normal as possible for your child. Encourage exercise and physical therapy. Find ways to make it fun. You can also help your child find a support group, so they can 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 to call your child’s healthcare provider

Call the healthcare provider if your child has any of these:

• Symptoms that don’t get better or get worse

• New symptoms