Scleroderma

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. 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.

Scleroderma is more common in women than men. Systemic scleroderma occurs more often in African-Americans and some Native Americans.

Symptoms

There are 2 forms of localized scleroderma:

  • Symptoms 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 often appear on your chest, stomach, and back, but also can occur on your face, arms, and legs. You may have 1 or more patches as small as half an inch or as large as 12 inches in diameter.
  • The main symptom is a line or band of skin that thickens and changes color. This line can appear on your arm, leg, or forehead. Linear scleroderma is more common in children.

There are 2 forms of systemic scleroderma:

  • Limited sclerodermaprogresses 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 also may have Raynaud’s disease or problems with frequent heartburn. Limited scleroderma also can affect your lungs, esophagus, and blood vessels.
  • Diffuse sclerodermaprogresses quickly. Symptoms include fatigue, loss of appetite, and joint swelling and pain. It can affect the skin all over your body, causing it to swell and become tight, shiny, and itchy. Over time, your skin may go back to normal. Diffuse scleroderma also can damage internal organs, such as your intestines, lungs, kidneys, and heart.

What causes scleroderma?

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

How is scleroderma diagnosed?

Your doctor will ask you about your symptoms and medical history. They also will perform a physical exam and look for changes in the appearance of your skin. Your doctor may want to remove a small sample of skin (biopsy) to look at it under a microscope. They also may 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

Your doctor 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, or dietary and lifestyle changes. Your doctor also may recommend physical or occupational therapy to help 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 doctor may work with specialists to treat your condition. For example, if scleroderma affects your heart, they may want to work closely with a cardiologist.

Questions

  • What type of scleroderma do I have?
  • What makes my scleroderma mild or severe?
  • What types of treatment will work best for me?
  • Are there any medicines I should take?
  • Do I need to see any specialists?
  • What are the possible complications of scleroderma?
  • Is scleroderma hereditary and can I pass it to my children?
  • Are there any support groups that you can recommend?

Can scleroderma be prevented or avoided?

You cannot prevent or avoid scleroderma.

Living with scleroderma

There is no cure for scleroderma. The best thing you can do is to work with your doctor(s) to manage symptoms and prevent complications. 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. Do not 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 doctor about other ways to help improve blood flow, including medicine. They also may prescribe medicines to treat skin patches, sores, and lesions.

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