Scleroderma and Kids
Parents and their children who have been diagnosed with scleroderma have a thirst for knowledge about the disease and available treatments. On this page and the others linked to it, we do our best to share important information.
Scleroderma isn’t easy to explain. It’s a complex disease. There are different forms of scleroderma. No one can predict who will get the disease and doctors can’t tell you exactly which symptoms you will experience, or how severe or mild those symptoms will be.
You can best use this information as a reference tool when talking with your pediatric rheumatologist. It can help you ask specific questions and share pertinent information so that you and your physician make the best decisions possible.
An overview of scleroderma and the differences between localized scleroderma and systemic sclerosis:
Scleroderma means “hard skin.” The cause and cure of scleroderma are unknown, but it is treatable. There are two main forms of scleroderma:
- Localized scleroderma (LS), also referred to as Morphea (primarily by dermatologists)
- Systemic sclerosis (SSc)
Localized scleroderma and systemic sclerosis are different diseases. Localized scleroderma does not turn into systemic sclerosis. Very rarely, children can have both types of scleroderma, either localized first or systemic first.
It’s common for both localized scleroderma and systemic sclerosis to have hard skin (cutaneous involvement), which is caused by inflammation and fibrosis.
The two forms of scleroderma also commonly have involvement of other tissues in addition to the skin (extracutaneous involvement). Most pediatric cases with LS and SSc have disease affecting skin and additional (extracutaneous) tissues. In both diseases, extracutaneous tissues that are commonly affected are fat, joints, tendons, muscles, and bone.
Symptoms that are different between LS and SSc are:
Skin involvement pattern:
LS skin involvement is usually limited to one limb, one side of the head, and/or one side of the body. In the affected body site, the involvement is usually not all one large lesion, but instead limited to certain parts of the site or ‘skipping’ in a line or band. Although typically found on one side of the body, it is not uncommon to affect both sides and multiple sites.
SSc skin involvement appears on both sides (bilateral). It tends to be circling around all sides of the limb (circumferential) and progresses from the hands, feet, and face to central parts of the body.
Extracutaneous (EC) pattern:
In LS, the affected extracutaneous tissues are usually located near to the skin lesion. The most common type involves muscle, tendons, fascia, and joint tissue (musculoskeletal). Children who have a lesion on the head can also have eye, mouth, and brain involvement.
In SSc, besides musculoskeletal involvement, inner organs such as the gastrointestinal tract, lung, heart, and kidneys can be affected. Whereas the gastrointestinal tract, lung, heart, and kidneys are rarely seriously affected in LS.
No two scleroderma journeys are the same, but there are common experiences along the way. No matter where you, your child, or a loved one are in your journey, or the type of scleroderma, the National Scleroderma Foundation can help you find your best path.