Besides thickening and hardening of the skin, scleroderma can cause skin to lose elasticity and become shiny as it stretches across bones. Other symptoms include:
- Numbness, pain or color changes in fingers, toes, cheeks, nose and ears, often brought on by cold or emotional distress
- Stiffness or pain in joints, such as elbows and knuckles
- Digestive problems ranging from not absorbing nutrients well to delayed movement of food because the muscles of the intestines aren't working well.
- Sores over joints, such as elbows and knuckles
- Puffy hands and feet, particularly in the morning
Causes and Risk Factors
About 150,000 Americans have scleroderma. It is a rare disease that affects women five times more often than men, and it affects adults more than children. It can run in families, but it more often occurs without any known family tendency. It is not considered contagious or cancerous.
Scleroderma results from the production of too much collagen, a fibrous protein in connective tissues. The body's immune system appears to attack the body, causing inflammation and overproduction of collagen. There are different types of scleroderma, including:
- Diffuse, which affects the skin of fingers, hands, arms, legs, face, neck and trunk. The lungs, heart, digestive tract and kidneys may be affected. It can interfere with the working of the digestive system, create breathing problems and cause kidney failure. Untreated, it may be fatal within several years.
- Limited, which involves the skin of fingers, lower arms and legs, face and neck. One variation is CREST syndrome. CREST stands for: 1) calcinosis, skin calcification; 2) Raynaud's phenomenon; 3) esophageal dysfunction, reflux or difficulty in swallowing; 4) sclerodactyly, hardening of the skin of the fingers or toes, and 5) telangiectasia, dilatation of tiny blood vessels, particularly of the skin.
- Localized, which affects mainly skin and deep tissues beneath the skin
Localized scleroderma includes the following types:
- Morphea, in which oval-shaped thick patches appear on the skin. The ovals are white in the middle with a purple border. These patches are most likely to occur on the torso but can also appear on arms, legs or forehead.
- Linear, in which bands or streaks of hardened skin appear on one or both of arms or legs or on the forehead.
- Overlap syndrome, which is a diffuse or limited systemic sclerosis with features of other connective tissue diseases. Mixed connective tissue disease (MCTD) is an overlap syndrome with features of scleroderma, lupus, polymyositis and rheumatoid arthritis. An antibody produced by the immune system that is directed against cellular proteins in the body is also present.
- Undifferentiated connective tissue disease, which has features of diffuse sclerosis, but there are no findings to make a definite diagnosis
- Sine scleroderma, which can be similar to either limited or diffuse scleroderma but does not affect the skin.