Scleroderma is a chronic skin disease which affects the skin’s connective tissue. It is also known as localized or systemic sclerosis. With scleroderma, the body produces an excess amount of collagen – the main structural protein in the connective tissue of the skin. Collagen is the most abundant protein in the human body and is responsible for holding different parts of the body together such as the muscles, skin, tendons, and bones. It’s estimated that about 300,000 people in the United States have scleroderma. 

How Scleroderma Forms

When the immune system produces too much collagen, the skin gets thick, tight, and hard. It also affects internal organs, blood vessels and digestive tract. Scars can form on the kidneys and lungs, causing tissue damage. Your blood vessels can thicken, leading to high blood pressure. 

There are two types of scleroderma: localized scleroderma and systemic scleroderma. Localized scleroderma affects the skin, while systemic scleroderma affects skin as well as multiple parts of the body. 

What causes scleroderma?

Scleroderma is caused by an excess amount of collagen by the immune system. It is unclear exactly what initiates the overproduction of collagen, except for that the immune system is involved. Scleroderma is known as an autoimmune disease. Our immune system normally protects us from foreign invaders like germs and bacteria. But with an autoimmune disease, the immune system actually attacks the body and damage its own tissues, causing inflammation. Other types of autoimmune diseases include lupus, multiple sclerosis, psoriasis, and Type 1 diabetes. 

Who is at risk for scleroderma?

Women are more affected by scleroderma than men. The disease most commonly occurs in people between the ages of 30 and 50. African-Americans and Native Americans have a higher risk for developing systemic scleroderma compared to European Americans. 

Signs and Symptoms of Scleroderma

The signs and symptoms of scleroderma can be different depending on which type of the disease you have and which parts of the body are affected by it. The most common signs and symptoms include:

• Tight and hard patches of skin which are usually in the shape of an oval or straight line. Depending on the type of scleroderma, the amount, size, and location of the patches may vary.
• Skin may look shiny where the patches of skin occur due to tightness. 
• Limited mobility in the area of the skin that is affected. 
• Small red spots on the face and chest (result of opened blood vessels)
• Swelling and pain in the fingers and/or toes
• Ulcers or sores on fingertips
• Weight Loss
• Shortness of breath
• Painful or swollen joints
• Muscle weakness
• Dry eyes or mouth 
• Heartburn
• Diarrhea
• Toes or fingers may be highly sensitivity to cold temperatures or emotional distress. This can cause pain, numbness, or loss of color in the toes and fingers. 
• Difficulty in the digestive system with absorbing nutrients. This happens if the intestinal muscles do not move food accordingly through the intestines. Acid reflux may also occur, which can cause damage to the esophagus. 
• Problems with the heart, lungs or kidneys, although this is rare. 

How is it diagnosed?

• Health assessment and review of medical history
• X-ray
• Blood tests
• Possibly a biopsy 
• Assessment of lungs, heart and esophagus 

Can scleroderma be treated?

Unfortunately, there is no treatment for scleroderma.However, there are a number of options available to control and manage the signs and symptoms of the disease. The most common forms of treatment involve medications, therapy, and surgery. It is possible for scleroderma to subside on its own within about three to five years. But with systemic scleroderma, which affects the internal organs, the disease often gets worse. 

• Heartburn medication can help alleviate symptoms of acid reflux
• Drugs that increase blood flow to your fingers
• NSAIDs for pain and swelling 
• Blood pressure medication to dilate the blood vessels, which may help prevent kidney and lung problems and prevent scar tissue from forming 
• Immune system suppressants such as steroids and other drugs to control your immune response, which can help with muscle, joint, or internal organ problems
• Antibiotic ointment to help prevent infections around the ulcers that may develop on the fingers
• Exercise 
• Light and laser therapy to treat the skin
• Physical therapy or occupational therapy
• Surgery, if scleroderma becomes severe and affects the internal organs
  • Organ transplant
    • Amputation, if ulcers develop into gangrene