Scleroderma

Overview

What is scleroderma?

Scleroderma (say: "sclare-oh-DER-muh") is a chronic (ongoing) disease that affects the skin and may also affect internal organs in some people. Scleroderma causes the body to produce too much collagen. Collagen is a protein that makes up connective tissues, such as the skin. Too much collagen can make the skin stretch, harden and thicken, and can also cause damage to internal organs, such as the heart, lungs and kidneys.

There are 2 types of scleroderma: localized and systemic. Localized scleroderma affects the skin only. Systemic scleroderma affects blood vessels and internal organs, as well as the skin.

Who gets scleroderma?

Anyone can have scleroderma, but women are more likely to develop it. Systemic scleroderma is more common in African-Americans and some Native Americans.

Symptoms

What are the symptoms of localized scleroderma?

There are 2 types of localized scleroderma:

  • Morphea. Symptoms may start with reddish patches of skin that thicken into hardened, oval-shaped areas. The patches later become whitish 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 half an inch and as large as 12 inches in diameter.

  • Linear. 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.

What are the symptoms of systemic scleroderma?

There are 2 types of systemic scleroderma:

  • Limited scleroderma progresses gradually. This type affects the skin of the fingers, hands, lower arms, legs and face. Like in localized scleroderma, patches of skin become thick and firm, and change color. People who have this type scleroderma may also have Raynaud’s disease. If their intestines are affected, they may have problems with frequent heartburn. Limited scleroderma may also affect the lungs, esophagus and blood vessels.

  • Diffuse scleroderma progresses quickly. People who have this type of scleroderma are often tired, 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?

Doctors don’t know what causes scleroderma. It is an autoimmune disease. Normally, antibodies produced by the immune system help protect the body against viruses, bacteria and other foreign substances. If you have an autoimmune disease, your immune system produces antibodies that attack your body’s tissues and/or organs.

Diagnosis & Tests

How can my doctor tell if I have scleroderma?

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

Treatment

How is scleroderma treated?

There is no cure for scleroderma. Localized scleroderma sometimes goes away on its own. Treatment usually focuses on relieving symptoms and preventing more damage to the skin and organs. Your doctor will choose the right treatment for you depending on your symptoms. If scleroderma is causing damage to internal organs, your doctor may work with other specialists to treat your condition. For example, if scleroderma is affecting your heart, your doctor may want to work closely with a cardiologist.

Your doctor may also recommend physical or occupational therapy to help you manage the pain.

Cosmetic surgery can sometimes help minimize the effects of scleroderma on your skin.

Questions to Ask Your Doctor

  • What kind of scleroderma do I have?

  • Will my scleroderma get worse?

  • Is there any treatment you can recommend to make me feel better?

  • Are there any medicines I should take?

  • Will I have any complications from scleroderma?

  • Should I have surgery?

  • Which of my symptoms are related to scleroderma?

  • Do I need to see any specialists?

  • Are my children more likely to get scleroderma?

  • Are there any support groups in my area?