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OVERVIEW

What is scleroderma?

Scleroderma is a rare autoimmune disorder made up of a group of diseases. There are 2 types of scleroderma: localized and systemic. Localized scleroderma affects the skin only. Systemic scleroderma affects the skin, as well as blood vessels and internal organs.

Scleroderma causes your body to produce too much collagen. Collagen is a protein that makes up connective tissues, such as the skin. When you have too much collagen, your skin can stretch, thicken, and harden. It also can cause damage to internal organs, such as the heart, lungs, and kidneys.

SYMPTOMS

What are the symptoms of localized scleroderma?

There are 2 types of localized scleroderma:

• Symptoms may start with red patches of skin that thicken into hard, oval-shaped areas. The patches later become white in the middle with purple borders. Patches usually appear on the chest, stomach and back, but can also affect the face, arms and legs. People who have this type of scleroderma may have 1 or more patches that can be as small as 1 cm (half an inch) and as large as 30 cm (12 inches) in diameter.
• This type of scleroderma causes a single line or band of skin that thickens and changes color. The line can appear on an arm, a leg or the forehead. Linear scleroderma is more common in children.

Within systemic scleroderma, there are 2 forms:

• Limited scleroderma progresses gradually. It affects the skin on your fingers, hands, lower arms, legs and face. It causes patches of skin to become thick and firm and change color. People who have this type may also have Raynaud’s disease problems with frequent heartburn. Limited scleroderma may also affect the lungs, esophagus and blood vessels.
• Diffuse scleroderma progresses quickly. Symptoms include fatigue, have little appetite and experience joint swelling and pain. It can affect the skin all over the body, causing it to swell, become shiny, tight and itchy. Eventually, skin becomes soft again and may go back to normal. Diffuse scleroderma may also damage internal organs, such as the intestines, lungs, kidneys and heart.

CAUSES & RISK FACTORS

What causes scleroderma?

The exact cause of scleroderma is unknown. It occurs when your immune system attacks your body’s tissues and/or organs.

DIAGNOSIS & TESTS

How can my healthcare provider tell if I have scleroderma?

Your healthcare provider will ask you about your symptoms and medical history. They will perform a physical examination and look for any changes in the appearance of your skin. Your healthcare provider may want to remove a tiny sample of your skin (biopsy) to examine it under a microscope. They may also order a blood test to check for antibodies or other tests to see if any internal organs have been affected. All of this helps to determine if you have scleroderma.

TREATMENT

How is scleroderma treated?

Your healthcare provider will choose the right treatment for you depending on your type and symptoms. Treatment focuses on relieving symptoms and preventing more damage. Possible treatments include medicine, such as creams for your skin. they may also recommend dietary and lifestyle changes. Your healthcare provider may suggest physical or occupational therapy to help you manage your pain. Cosmetic surgery may help to lessen the effects of scleroderma on your skin.

Localized scleroderma sometimes goes away on its own. If scleroderma has caused internal damage, your healthcare provider may work with specialists to treat your condition. For example, if scleroderma affects your heart, your healthcare provider will work closely with a heart specialist (cardiologist).

Living with scleroderma

There is no cure for scleroderma. The best thing you can do is to work with your healthcare provider(s) to manage symptoms and prevent complications. Plus, there are things you can do on your own that may help.

If you have skin problems, try to keep your skin soft and moisturized. Apply lotions often, wear sunscreen outside, and use a humidifier at home. Avoid harsh skin products, as well as hot showers and baths.

If you have heartburn or other digestive problems, try to eat small, frequent meals. Don’t lay down right after eating. Avoid spicy foods, alcohol, caffeine, and other things that trigger heartburn.

• If you have Raynaud’s disease, you can relieve symptoms by:
• Not smoking
• Wearing warm clothes, including socks and gloves, if needed
• Doing regular exercise
• Avoiding things that cause increased stress or anxiety

Talk to your healthcare provider about other ways to help improve blood flow, including medicine. They may also prescribe medicines to treat skin patches, sores, and lesions.

Be sure to contact your healthcare provider if you have new or worsening symptoms or health problems.