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 degenerative and gets worse over time. The disease can be in one area of the body. This is known as localized scleroderma. Or it may affect the whole body. This is known as systemic sclerosis. Systemic sclerosis occurs only rarely in children. Localized scleroderma is seen more often in children. It may involve patches of the skin on the trunk, arms, legs, or head.
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:
- Thickening and swelling of the tips of the fingers
- Pale and tingly fingers that may turn numb when exposed to 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
- Discolored (lighter or darker) skin
- Joint tightness
The symptoms of scleroderma can be like other health conditions. Make sure your child sees his or her health care provider for a diagnosis.
How is scleroderma diagnosed?
The process starts with a medical history and 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. Tests may also be done, such as:
Electrocardiogram (ECG). This test records the electrical activity of the heart, shows abnormal rhythms, and detects 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 is done to look 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?
Treatment will depend on your child's symptoms, age, and general health. Treatment may include:
- Nonsteroidal anti-inflammatory medicines or corticosteroids, to relieve pain
- Penicillamine, to slow the skin thickening process and delay damage to internal organs
- Immunosuppressive medicines, such as methotrexate
- Treatment of specific symptoms, such as heartburn and Raynaud's phenomenon
- Physical therapy and exercise, to maintain muscle strength
Talk with your healthcare providers 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 complications. Possible complications may include:
- Thickened, tight skin
- Behavior and learning problems
- Vision changes
- Gastroesophageal reflux
- Less respiratory function
- Heart and kidney damage
Helping your child live with scleroderma
Work with your child's health care 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 with 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?
If your child's symptoms get worse or he or she has new symptoms, let the healthcare provider know.
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.
Tips to help you get the most from a visit to your child’s healthcare provider:
- Before your visit, write down questions you want answered.
- At the visit, write down the names of new medicines, treatments, or tests, and any new instructions your provider gives you for your child.
- 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.
- Fetterman, Anne, RN, BSN
- Freeborn, Donna, PhD, CNM, FNP