Causes of Pediatric Localized Scleroderma

What Causes Localized Scleroderma?

Our immune system protects us from getting sick. It clears out germs that may enter our body. Inflammation is a natural, healthy part of that process, but it’s usually short-lived.

In localized scleroderma, also called morphea, the immune system doesn’t work correctly. Inflammation doesn’t go away. It keeps going, inflaming the skin and often, the tissues under the skin. This sets off fibrosis: scarring of skin and tissues. Localized scleroderma is called an autoimmune disease.

Our immune system has many parts and players. The two main parts are the innate immune system (fast responders) and adaptive immune system (slower, unique, targeted).

Our adaptive immune system:

  • Kills infected cells
  • Lowers or raises inflammation
  • Makes antibodies and other cells

When a healthy immune system reacts to a common virus like the cold or flu, it sends its disease-fighting messengers to attack and wipe out the threat.

If you have an autoimmune disease, your adaptive immune system usually clears out the virus. But your cells don’t calm down after the attack. They stay on high alert. They may attack normal, healthy tissues in your body.

It’s unclear why this happens. Some people may be born with certain genes that make them more likely to develop an autoimmune disease. Others may be exposed to something in the environment, like an infection or smoking, that ‘turn on’ the autoimmune reaction. Researchers are trying to find out why this happens in some people, but not others.

In localized scleroderma, your immune system attacks the skin and tissue underneath as the ‘foreign object.’ You may build up extra immune cells and inflammation in these areas of skin and tissue that shouldn’t be there. Your skin changes. After some time, these skin changes may become like a scar.

Inflammation is your body’s normal repair system. But inflammation that doesn’t stop sets off a new chain of events. Skin cells make collagen, a normal repair protein. Normally, extra collagen is cleaned up when the repair is done, but in localized scleroderma, your skin keeps making more and more collagen. You may have a thick build-up of scar-like collagen. You may lose healthy cells in your skin, muscle, bone, and other tissues.

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Where to Start Find Your Path

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.