Scleroderma is an autoimmune disease that sometimes only affects the skin, but it can affect internal organs as well. This rare and chronic disease triggers the immune system to mistakenly attack the body’s own tissues. The result is inflammation and the production of too much collagen, the most abundant protein in the body. Collagen is found in the skin, connective tissues, organs, blood vessels and other parts of the body, and it is the main component of scar tissue.
Because collagen is found in so many places, its overproduction can cause a wide range of symptoms all over the body. It is estimated that 300,000 people in the United States live with the various forms of scleroderma and up to 10,000 a year die from it.
Symptoms vary depending on the type of scleroderma and even from person to person within the same type. Localized scleroderma, one of the two main types of the disease, affects the skin and related tissues. It causes thick, hard patches or lines on the skin, which may spread.
The second main type is systemic scleroderma, or systemic sclerosis. It affects internal organs and blood vessels as well as the skin, and is the more serious form.
General symptoms for scleroderma include:
- Skin-related signs. Skin gets thicker, tighter, and harder. It takes on a shiny, glossy, or waxy appearance. Color changes may occur. Telangiectasia, caused by small blood vessels swelling in the face and hands, results in red spots on skin.
- Raynaud’s phenomenon. This occurs when small blood vessels in the hands and feet spasm when exposed to cold or stress. That blocks blood circulation and causes fingertips to turn white or blue and become painful or numb. They may turn red when the hands are warm again.
- Digestive problems. Esophageal dysfunction is common with systemic scleroderma. It causes smooth muscles in the esophagus to have trouble moving food. The disease can attack any part of the gastrointestinal tract, causing heartburn, nausea, vomiting, constipation, diarrhea and other symptoms.
- Heart and lung problems. If scleroderma affects the lungs, it can cause difficulty breathing due to scarring from skin thickening. It also may cause an abnormal heartbeat, high blood pressure, and heart failure.
Causes and Risk Factors
Scleroderma is more common in women than men. Other risk factors involve:
- Immune system. Scleroderma is viewed as an autoimmune disease. People with scleroderma often have symptoms of other autoimmune diseases like lupus, rheumatoid arthritis, or Sjögren’s syndrome.
- Genetics. The disease is not passed down from parent to child, but specific genetic variations are associated with an increased risk. In some families and ethnic groups, scleroderma appears more often.
- Environmental triggers. Repeated exposure to harmful substances or chemicals seems to increase the risk of getting this disease. Some viruses, medications, and drugs may also trigger symptoms
Problems from scleroderma can be anywhere from mild to severe, affecting any or all of these:
- Fingertips. Restricted blood flow may cause damage to fingertip tissues, resulting in pits and sores. If poor blood supply continues, fingertip tissues may even die.
- Lungs. Scarring may occur and make breathing difficult. This can reduce the patient’s ability to exercise and engage in normal activities. High blood pressure and pneumonia also may occur.
- Kidneys. A sudden increase in blood pressure can cause kidney failure.
- Heart. Abnormal heartbeats and congestive heart failure may result from scarring of heart tissue. The disease also may cause inflammation of heart tissues.
- Teeth. If facial skin tightens too much, it can shrink the opening of the mouth. That makes it harder to eat and to care properly for the teeth. Normal saliva production may be reduced as well, increasing problems with tooth decay.
- Digestive system. Heartburn and trouble swallowing are typical problems. Cramps, bloating, and both constipation and diarrhea can occur. The effects of scleroderma also can make it harder to absorb nutrients.
- Joints. Thickening skin, particularly in the hands, can become so tight that it restricts the flexing of joints.
Diagnosis and Treatment
Because scleroderma is rare and has so many forms and symptoms, diagnosis can be difficult and time consuming. Once it has been diagnosed, many forms of treatment are used to try to control and mitigate the symptoms.
- Lifestyle choices. Working in conjunction with a doctor, people can ease symptoms by taking such measures as staying active, using lotions and sunscreen to protect skin, quitting smoking, protecting themselves from the cold, and eating in a way that manages heartburn and other digestive trouble.
- Medication. Drug strategies include the use of anti-inflammatory drugs, immunosuppressants, corticosteroids, and blood pressure medications.
- Therapies. Physical therapy, occupational therapy, and psychotherapy may all be recommended for the problems caused by scleroderma. In addition, different forms of light therapy are used to ease skin symptoms.
- Stem cell and organ transplants. Stem cell transplants may be used in severe cases to try to regenerate tissue. If the damage to lungs or kidneys is bad enough, organ transplants may be performed
Help with Scleroderma
The Florida Medical Clinic Department of Rheumatology offers consultative, diagnostic and therapeutic services to help you reclaim your quality of life. Our areas of expertise include treating scleroderma, osteoporosis, osteoarthritis, psoriatic arthritis, and rheumatoid arthritis. Contact us today to schedule an appointment.
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