Scleroderma: Main Types & Top Questions Answered

Scleroderma is a chronic skin disease, meaning it doesn’t go away. Your doctor can treat your symptoms and help you feel better, though.

The problem is with your immune system, which causes your body to make too much of the protein collagen, an important part of your skin.

As a result, your skin gets thick and tight, and scars can form on your lungs and kidneys. Your blood vessels can thicken and not work the way they should. This leads to tissue damage and high blood pressure.

There are two types:

Localized scleroderma mainly affects the skin.

There are two kinds of localized scleroderma:

  • Morphea: This involves hard, oval-shaped patches on the skin. They start out red or purple and then turn whitish in the center. Sometimes, but not often, this type can affect blood vessels or internal organs. This is called generalized morphea.
  • Linear: This kind causes lines or streaks of thickened skin to form on the arms, legs, or face.

Systemic scleroderma, also called generalized scleroderma, can involve many body parts or systems. There are two kinds of this as well:

  • Limited scleroderma: It comes on slowly and affects the skin of the face, hands, and feet. It can also damage the lungs, intestines, or esophagus, the tube that carries food from your mouth to your stomach. This is sometimes referred to as CREST syndrome.

For many people with limited scleroderma, the outlook is good, but the disease tends to get worse over time. Sometimes, it can affect the heart and raise blood pressure in the lungs -- though this can be treated.

  • Diffuse scleroderma: This comes on quickly. Skin on the middle part of the body, thighs, upper arms, hands, and feet can become thick. This form also affects internal organs, like the heart, lungs, kidneys, and gastrointestinal tract.

What Causes Scleroderma?

Doctors don’t know what triggers scleroderma. It’s one of a group of conditions known as autoimmune diseases. These happen when your immune system, which usually protects you from germs, turns on your body and causes inflammation of skin and other organs.

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What Are the Symptoms of Scleroderma?

The symptoms can affect many parts of your body. They include:

  • Hardened or thickened skin that looks shiny and smooth. It’s most common on the hands and face.
  • Cold fingers or toes that turn red, white, or blue. This is called Raynaud's phenomenon.
  • Ulcers or sores on fingertips
  • Small red spots on the face and chest. These are opened blood vessels called telangiectasias.
  • Puffy or swollen or painful fingers and/or toes
  • Painful or swollen joints
  • Muscle weakness
  • Dry eyes or mouth (called Sjogren's syndrome)
  • Swelling -- mostly of the hands and fingers. Your doctor may call this edema.
  • Shortness of breath
  • Heartburn
  • Diarrhea
  • Weight Loss

How Is Scleroderma Diagnosed?

Your doctor will check you and ask about your health history. He’ll likely take an X-ray, do some blood tests, or take a small sample of skin (called a biopsy). He may check out your heart, lungs, and esophagus.

How Is Scleroderma Treated?

There’s no treatment for scleroderma, but you can manage the symptoms. Your doctor will focus on helping you do that with:

  • NSAIDs (nonsteroidal anti-inflammatory drugs like ibuprofen or aspirin). They can help with swelling and pain.
  • Steroids and other drugs to control your immune response. These can help with muscle, joint, or internal organ problems.
  • Drugs that boost blood flow to your fingers
  • Blood pressure medication
  • Drugs that open blood vessels in the lungs or prevent tissue from scarring
  • Heartburn medication

Other things that help may include:

WebMD Medical Reference Reviewed by Arefa Cassoobhoy, MD, MPH on July 14, 2014

Sources

SOURCES: 

National Institute of Arthritis and Musculoskeletal and Skin Diseases. 

Scleroderma: Etiology, Goldman: Cecil Medicine, 24th ed.

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