Scleroderma, also known as systemic sclerosis, is a group of rare diseases that involve the hardening and tightening of the skin. It also may cause problems in the blood vessels, internal organs and digestive tract.

About 75,000–100,000 people in the U.S. have scleroderma, according to the American College of Rheumatology, and it occurs much more often in women than in men.

Scleroderma often is categorized as "limited" or "diffuse," which refers only to the degree of skin involvement. Both types can involve any of the other vascular or organ problems. Localized scleroderma, also known as morphea, affects only the skin.

Signs and symptoms

The signs and symptoms of scleroderma vary from person to person, depending on which parts of the body are affected.

Nearly everyone who has scleroderma experiences a hardening and tightening of the skin. Early symptoms may include swelling and itchiness. Affected skin can become lighter or darker in color and may look shiny because of the tightness. Some people also experience small red spots, called telangiectasia, on their hands and face. Calcium deposits can form under the skin, particularly at the fingertips, causing bumps that can be seen on X-rays.

Raynaud's phenomenon is common in scleroderma and occurs because of an exaggerated contraction of the small blood vessels in the fingers and toes in response to the cold or emotional distress. When this happens, the skin may turn white, blue or red, and feel painful or numb.

Scleroderma can affect any part of the digestive system, from the esophagus to the rectum. Depending on which parts of the digestive system are affected, signs and symptoms may include heartburn, difficulty swallowing, bloating, diarrhea, constipation and fecal incontinence.

When scleroderma affects the heart or lungs, it can cause shortness of breath, decreased exercise tolerance and dizziness. Scleroderma can cause scarring in the lung tissues that may result in increasing shortness of breath over time. Scleroderma also can cause the blood pressure to increase in the circulation that goes between the heart and the lungs. This is called pulmonary hypertension. When scleroderma affects the heart, heartbeats can become irregular. Heart failure also can occur in some people.

Treating scleroderma

No treatment can cure or stop the overproduction of collagen that is characteristic of scleroderma. But there are treatments that can control symptoms and prevent complications, including medications and physical therapy. Stem cell transplants might be an option for people who have severe symptoms that haven't responded to treatment. If the lungs or kidneys have been severely damaged, organ transplants might be considered.

Lifestyle and home remedies

These strategies can manage your symptoms of scleroderma:

• Stay active.
  Exercise keeps your body flexible, improves circulation and relieves stiffness. Range-of-motion exercises can keep your skin and joints flexible. This is always important, but especially early in the disease course.
• Protect your skin.
  Take good care of dry or stiff skin by using lotion and sunscreen regularly. Avoid hot baths and showers and exposure to strong soaps and household chemicals, which can irritate and further dry out your skin.
• Don't smoke.
  Nicotine causes blood vessels to contract, worsening Raynaud's disease. Smoking also can cause permanent narrowing of the blood vessels, and cause or exacerbate lung problems.
• Manage heartburn.
  Avoid foods that give you heartburn or gas. Also avoid late-night meals. Elevate the head of your bed to keep stomach acid from backing up into your esophagus as you sleep. Antacids may relieve symptoms.
• Protect yourself from the cold.
  Wear warm mittens for protection anytime your hands are exposed to cold, even when you reach into a freezer. It is also important to keep your core body temperature warm to prevent Raynaud's symptoms. When you're outside in the cold, wear warm boots, cover your face and head, and wear layers of warm clothing.

Connect with others talking about living with scleroderma in the Autoimmune Diseases support group on Mayo Clinic Connect, an online patient community moderated by Mayo Clinic.

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