Scleroderma

What Is Scleroderma?

Scleroderma is a long-lasting disease that affects your skin, connective tissue, and internal organs. It happens when your immune system 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 may thicken and stop working the way they should. This leads to tissue damage and high blood pressure.

Scleroderma isn’t contagious or infectious, meaning you can’t get it from other people. There’s no cure, but treatment can ease your symptoms so you feel better.

Types of Scleroderma

There are two kinds of scleroderma:

Localized scleroderma mainly affects your skin. It happens in one of two forms:

  • Morphea. This involves hard, oval-shaped patches on your skin. They start out red or purple and then turn whitish in the center. Sometimes, this type can affect blood vessels or internal organs. This is called generalized morphea.
  • Linear. This kind causes lines or streaks of thickened skin on your arms, legs, or face.

Systemic scleroderma, also called generalized scleroderma, can involve many body parts or systems. There are two types:

  • Limited scleroderma. This comes on slowly and affects the skin of your face, hands, and feet. It can also damage your lungs, intestines, or esophagus, the tube that carries food from your mouth to your stomach. It’s sometimes called CREST syndrome, after its five common signs:
    • Calcinosis. This is when calcium salts form nodules under your skin or in your organs.
    • Raynaud’s phenomenon. This is a lack of blood flow to parts of your body such as your fingers, toes, or nose, usually because of cold. Your skin might turn red, white, or blue.
    • Esophageal dysfunction. This is when your esophagus doesn’t work the way it should.
    • Sclerodactyly. This is when your skin becomes thin and shiny. It usually causes problems with moving your fingers and toes.
    • Telangiectasia. This is when small blood vessels grow near the surface of your skin.

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

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Scleroderma Causes

Doctors don’t know what causes 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, instead causes inflammation of your skin and other organs.

Scleroderma Risk Factors

Anyone can get scleroderma. It usually happens in women and in people who are ages 35 to 55. Other things that might raise your risk include:

  • Certain changes in your genes
  • A family history of autoimmune disease
  • Triggers in the world around you like viruses, medications, or chemicals

Scleroderma Symptoms

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 your hands and face.
  • Raynaud's phenomenon
  • Ulcers or sores on your fingertips
  • Small red spots on your face and chest
  • Firm, oval-shaped patches on your skin
  • Trouble swallowing
  • Painful or swollen joints
  • Muscle weakness
  • Dry eyes or mouth (Sjogren's syndrome)
  • Swelling, mostly of your hands and fingers (edema)
  • Shortness of breath
  • Belly cramps and bloating
  • Heartburn
  • Diarrhea
  • Weight loss with no clear cause

Scleroderma Diagnosis

Your doctor will do a physical exam and ask about your health history. They might order tests including:

  • Imaging tests such as X-rays and CT scans
  • Blood tests
  • Gastrointestinal tests
  • Lung function tests
  • Heart tests such as EKGs and echocardiograms

They might also take a small sample of skin (called a biopsy) for a specialist to look at under a microscope.

Scleroderma Complications

Treatment can help lower your risk of complications, which may include:

  • High blood pressure in your lungs
  • Scar tissue in your lungs
  • Loss of blood flow to your fingers and toes
  • Muscle inflammation
  • Infection
  • Kidney failure

Scleroderma Treatment

You can manage the symptoms of scleroderma with:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or aspirin. They can help with swelling and pain.
  • Steroids and other medications to slow your immune system. These can help with muscle, joint, or internal organ problems.
  • Drugs to boost blood flow to your fingers
  • Blood pressure medication
  • Drugs to open blood vessels in your lungs or to keep tissue from scarring
  • Heartburn medication
  • Antibiotics
  • Medications to help move food through your intestines

Other things that help may include:

WebMD Medical Reference Reviewed by Stephanie S. Gardner, MD on March 06, 2020

Sources

SOURCES: 

National Institute of Arthritis and Musculoskeletal and Skin Diseases.

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

UpToDate: "Overview of the treatment and prognosis of systemic sclerosis (scleroderma) in adults."

Mayo Clinic. "Scleroderma."

Scleroderma Foundation. "What is scleroderma?"

American College of Rheumatology: “Scleroderma.”

National Organization for Rare Disorders: “Scleroderma.”

Cleveland Clinic: “Scleroderma: An Overview,” “4 Questions You May Have About Scleroderma.”

National Library of Medicine Genetics Home Reference: “Systemic scleroderma.”

Johns Hopkins Scleroderma Center: “Understanding Scleroderma.”

Medscape: “Scleroderma.”

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