Scleroderma is a rare autoimmune disorder that causes abnormal growth of skin and other connective tissue. The term, derived from Greek, means “hard skin” and refers to the thickening and tightening of the skin and inflammation and scarring of other organs, including the heart, liver, and kidneys.Scleroderma is relatively rare. According to the American College of Rheumatology, there are about 75,000 to 100,000 people in the U.S. who have this disease, mostly women between the ages of 30 and 50.
Types of Scleroderma
There are two types of scleroderma – localized scleroderma that affects only certain parts of the body, and systemic sclerosis that affects the whole body.Localized Scleroderma: Localized scleroderma mainly affects your skin, although it can spread to your muscles, joints, and bones. It happens in two forms:
- Morphea – Symptoms of morphea include hard, oval-shaped discolored patches on the skin. They start out red or purple and then turn whitish in the center. It sometimes affects blood vessels and internal organs, called generalized morphea.
- Linear Scleroderma – Streaks or bands of thick, hard skin on the arms, legs, or face is called linear scleroderma. When it occurs on the face and forehead, it is called en coup de sabre.
Systemic Scleroderma: is the most serious form of the disease and is sometimes called generalized scleroderma. It can involve many body parts or systems, including skin, muscles, joints, lungs, kidneys, and the heart. There are two major forms of systemic scleroderma, limited systemic sclerosis, and diffuse scleroderma.
- Limited Scleroderma – This comes on slowly and affects the skin of your face, hands, and feet, as well as potentially damaging your lungs, intestines, and esophagus. It is sometimes called CREST syndrome.
- Diffuse Scleroderma – This type of scleroderma comes on quickly. The skin on the middle part of your body, thighs, upper arms, hands, and feet can become thick. People with diffuse scleroderma often are tired, lose appetite and weight, and have joint swelling and pain. People with diffuse scleroderma face the most serious long-term outlook if they develop severe kidney, lung, digestive, or heart problems. Fortunately, less than one-third of patients with the diffuse disease develop these severe problems. Early diagnosis and continual and careful monitoring are important.
Our caring Providers can come up with an individualized treatment plan and help in monitoring your scleroderma.