About Scleroderma
What is scleroderma?
Scleroderma is a rare disease that causes skin thickening, hardening and tightness. Scleroderma also may involve internal organs, leading to intestinal, kidney, lung and heart problems. It is a chronic disease, which means it lasts a long time.
When scleroderma only involves the skin, it is called localized scleroderma. When there is internal organ involvement, it is called generalized scleroderma or systemic sclerosis . Early diagnosis and treatment are essential to help prevent many of the complications of scleroderma.
What are some of the symptoms of scleroderma?
Common skin symptoms include:
- Thickening, hardening and tightness
- Swelling of hands and toes
- Hair loss in involved skin
- Sores over fingertips
- Generalized itching
- Color changes of hands and feet with cold exposure (known as Raynaud’s)
Other common symptoms include:
- Fatigue
- Arthritis
- Muscle pain and weakness
- Dryness of the eyes and mouth
- Heartburn, bloating and other digestive symptoms
- Shortness of breath
- Cough
- High blood pressure (known as hypertension)
Who gets scleroderma?
Scleroderma is a rare disease. Approximately 10-20 new cases per million people are diagnosed each year. People of all races and ethnic backgrounds get scleroderma, yet approximately 75 percent of people with the disease are women. It can occur at any age. However, scleroderma most commonly begins when people are between the ages of 30 and 60 years old.
What causes scleroderma?
The cause of scleroderma is not known.
We do know that scleroderma is an autoimmune disease. This means the body’s natural immune system does not behave normally. Instead of serving to fight off infections from bacteria, viruses and the like, the immune system of a person with scleroderma attacks its own body. This damages blood vessels.
The body’s response to damage to its blood vessels is to make specific proteins, called collagen. The collagen repairs the damage by forming diffuse scars (fibrosis) throughout the blood vessels. The excess collagen can deposit in the skin. This causes the many skin changes seen with scleroderma. In addition, the fibrosis of the blood vessels leads to derangements of many of the internal organs.
This information has been developed and approved by Aryeh Fischer, MD (April 2008).
