Systemic scleroderma is a rare autoimmune disease affecting children that causes thick, dense scar tissue to replace normal tissue. It can cause symptoms that affect your child’s skin and internal organs. Medications and physical therapy are potential treatments that can help.
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Juvenile systemic scleroderma (systemic sclerosis) is a condition that causes your child’s skin to become unusually thick and hard. It may also cause symptoms that affect their organs. Systemic means that the condition affects your child’s entire body.
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Cleveland Clinic is a non-profit academic medical center. Advertising on our site helps support our mission. We do not endorse non-Cleveland Clinic products or services. Policy
Systemic scleroderma is an autoimmune disease. This condition causes your child’s immune system to attack their body’s healthy cells. Scleroderma creates inflammation (swelling) in the skin. The swelling triggers an overproduction of collagen cells, a protein that gives tissues support and contributes to your child’s flexibility. Too much collagen causes fibrosis, or scarring, in your child’s skin.
There are three types of systemic scleroderma, which include:
Juvenile systemic scleroderma can affect anyone of any age or sex, but it’s rare in early childhood. The term “juvenile” means that the condition affects children. There are about 5,000 to 7,000 children with scleroderma in the United States. Only 2% of all people with scleroderma develop the disease before age 10, and 7% develop it between ages 10 and 19.
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Juvenile systemic scleroderma causes symptoms that affect your child’s skin and organs. Children with systemic scleroderma also typically experience Raynaud’s phenomenon.
Symptoms of systemic scleroderma that affect your child’s skin and tissues include:
Initially, the skin of the hands and feet appear swollen (bigger). Over time, your child's skin tightens and hardens and may appear to have ridges, depressed areas or small pits.
Systemic scleroderma affects your child’s internal organs and could cause symptoms that include:
Pain isn’t usually severe, so it’s common for children to have scleroderma without knowing about it.
Children diagnosed with systemic sclerosis should see their healthcare provider often to check for high blood pressure and lung, kidney, gastrointestinal and heart problems.
Raynaud’s phenomenon causes white, blue and red color changes to your child’s fingers and/or toes when they're exposed to cold or stress. This condition is present early in the disease’s course and may be a sign of systemic sclerosis.
Other symptoms of Raynaud’s phenomenon include:
The exact cause of juvenile systemic scleroderma is unknown. Research suggests an injury to the cells that line blood vessels, which then over-activates the skin’s connective tissue cells (fibroblasts), causes the condition. Fibroblasts produce proteins that include collagen, and a buildup of collagen causes symptoms of systemic scleroderma.
With systemic scleroderma, too much collagen in your child’s tissues causes their skin to become hard. The immune system helps defend the body against infection or illnesses. For children diagnosed with scleroderma, their immune system overreacts and defends itself from their body’s healthy cells. This causes your child’s cells to produce too much collagen (protein). The extra collagen deposits in your child’s skin and organs and causes hardening and thickening (similar to the scarring process).
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Your child’s healthcare provider will diagnose systemic scleroderma based on several factors, including:
Lab tests help eliminate other conditions that are similar to systemic scleroderma, assess how active scleroderma is and determine if there are organs other than the skin involved. Testing for autoimmune proteins can help determine the course of the condition. Rarely do physicians perform a skin biopsy.
Additional tests for systemic scleroderma include:
Your child's healthcare provider might team up with a rheumatologist (arthritis/autoimmune specialist) or dermatologist (skin specialist) and other specialists that are experts in specific areas of the body (GI tract, heart, lungs and kidneys) to diagnose, monitor and treat systemic scleroderma.
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The objective of systemic scleroderma treatment is to stop inflammation, keep the condition from getting worse and prevent internal organ involvement.
Protecting the skin will help maximize blood flow to the skin, hands and feet, especially for children who have Raynaud's phenomenon. Tips to protect your child’s skin include:
Basic stretching and guided exercise programs with physical and occupational therapists help your child maintain flexibility, joint range of motion, muscle strength and blood flow to the affected areas. Therapy will also help prevent joint contractures (bends at the joints). Your provider might recommend splints if necessary.
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In rare cases, orthopedic hand or cosmetic surgery may be necessary to correct severe joint or skin deformities or scars. Before surgery, the condition must be in remission (not active) for several years.
Your child's healthcare provider might consider autologous bone marrow transplantation as a treatment option. Other treatments are currently under investigation.
Your child’s healthcare provider might recommend different medicines to treat their diagnosis, including:
There's no way to prevent systemic scleroderma because the cause is unknown.
There's no cure for scleroderma, but you can help your child manage their condition.
Systemic scleroderma is a chronic (long-term) and slowly developing disease, lasting for months or years. Your child's outlook depends on how widespread their symptoms are and how much of their body (especially their skin and internal organs) is involved.
Children with lung, heart or kidney problems have the greatest risk for complications, including joint contractures and changes to their physical appearance (cosmetic changes). The condition can affect your child’s bone growth.
Although systemic scleroderma often doesn’t go away, it can remain at the same level without getting worse for several years.
The life expectancy for a child diagnosed with systemic scleroderma varies based on the severity of their condition, especially if it affects their organs.
Children with systemic scleroderma should live life as normally as possible. They should attend school, play sports and participate in activities. In general, there are no limitations to the physical activities children can do (as long as they're safe). Exercise will help prevent deconditioning and increase muscle strength, flexibility and endurance.
Your child should visit their provider regularly for checkups, especially to monitor for signs and symptoms that affect their internal organs. If your child experiences severe pain, has difficulty moving parts of their body or has symptoms that affect their day-to-day life, visit their provider immediately.
There are two types of scleroderma that are different from the types of systemic scleroderma. These types include:
A note from Cleveland Clinic
Although systemic scleroderma targets your child’s entire body, you can make efforts to preserve your child’s ability to have a normal childhood whenever possible. As your child’s caregiver, make sure to stay up to date on checkups to monitor your child’s diagnosis and treatment. If your child has trouble moving, playing or participating in activities, talk to your child's healthcare provider.
Last reviewed on 08/05/2022.
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