What is Scleroderma

Scleroderma is a rare and long-term (chronic) disease of the immune system. The immune system protects the body by attacking germs that cause illness.

If you have scleroderma, your immune system mistakenly attacks your skin and other parts of your body instead. This is called an autoimmune disease.

Scleroderma means hardening of the skin. If you have a mild form of this condition, it may affect only your skin (localized scleroderma).

If you have a severe form, it may affect your skin and also your blood vessels, lungs, kidneys, heart, and digestive system (systemic scleroderma).

What are the causes?

The cause of this condition is not known.

What increases the risk?

You are more likely to develop this condition if:

• You have a family history of scleroderma.
• You are female.
• You are 30–50 years old.

What are the signs or symptoms?

Symptoms of this condition depend on the type of scleroderma you have. They also vary from person to person. Symptoms of localized scleroderma may include:

• Discolored patches of skin (morphea). These may be thick and waxy.
• Bands of thick, hard skin on your arms, legs, or face (linear scleroderma).
• Tightening of the skin that limits how well you can move your joints.
• Open skin sores.

Symptoms of systemic scleroderma may include:

• Discoloration of the fingers and sometimes the toes (Raynaud’s phenomenon). Your fingers or toes may turn blue, white, or red. You may also have tingling or numbness. Exposure to cold often triggers this symptom.
• Tightening of the skin of the fingers, hands, arms, neck, and face.
• Enlarged blood vessels of the hands, face, and nail beds (telangiectasias).
• Calcium deposits under your skin (calcinosis).
• Joint pain.
• Heartburn.
• High blood pressure.
• Constipation.
• Trouble swallowing (dysphagia).
• Trouble breathing.

How is this diagnosed?

This condition may be diagnosed based on:

• Your symptoms and medical history.
• A physical exam.
• Tests, such as:
  • Imaging studies. This may include X-rays and CT scan.
  • Blood tests.
  • Lung (pulmonary) function tests.

Scleroderma can be hard to diagnose because other diseases have many of the same symptoms. You may need to see specialists as directed by your health care provider.

How is this treated?

There is no cure for this condition, but treatment can relieve symptoms and prevent complications. Mild symptoms may not need treatment. Treatment may include taking medicines to:

• Improve blood flow.
• Block production of stomach acid to treat heartburn.
• Treat high blood pressure caused by kidney disease.
• Relieve joint pain and inflammation.
• Treat lung symptoms.

Follow these instructions at home:

Medicines

• Take over-the-counter and prescription medicines only as told by your health care provider.
• Do not drive or use heavy machinery while taking prescription pain medicine.

Eating and drinking

• Eat a healthy diet.
• Drink enough fluid to keep your urine pale yellow.
• If you have heartburn:
  • Eat smaller meals often.
  • Avoid spicy and fatty foods.
  • Do not eat meals late in the evening.
  • Avoid lying down right after you eat.

Lifestyle

• If you have Raynaud’s phenomenon, cold can trigger your symptoms. To prevent symptoms, you can:
  • Wear mittens, a hat, a scarf, and warm footwear.
  • Dress in layers during cold weather.
  • If possible, stay indoors during cold weather.
• Wear comfortable shoes that are well cushioned.
• Protect your skin with sunscreen and moisturizers.
• Stretch and exercise regularly.
• Maintain a healthy weight.

General instructions

• Learn as much as you can about scleroderma, and work closely with your team of health care providers.
• Do not use any products that contain nicotine or tobacco, such as cigarettes and e-cigarettes. If you need help quitting, ask your health care provider.
• Ask your health care provider how to check your blood pressure at home. Check it as directed by your health care provider.
• Make sure you have a good support system at home.
• Use skin moisturizer to keep your skin moist.
• Keep all follow-up visits as told by your health care provider. This is important.

Contact a health care provider if:

• Your scleroderma symptoms change or become worse.
• You have concerns about your mental health.

Get help right away if:

• A finger or toe becomes painful or numb.
• A finger or toe turns black or a very dark color.
• You have:
  • Swelling, pain, or tenderness in an arm or leg.
  • Trouble swallowing.
  • Trouble breathing.
  • Chest pain.
• You cough up blood.

Summary

• Scleroderma is a rare and long-term (chronic) disease of the immune system. If you have scleroderma, your immune system mistakenly attacks your skin and other parts of your body.
• Symptoms of this condition depend on the type of scleroderma you have. They also vary from person to person.
• There is no cure for this condition, but treatment can relieve symptoms and prevent complications.