Scleroderma is a rare disease of the connective tissue. It can cause the tissue in skin, joints, and internal organs to thicken and stiffen. There are three major forms of the disease:
Localized scleroderma (also known as morphea)—usually affects only the skin in isolated parts of the body. This form is less serious.
Systemic scleroderma—affects widespread areas of skin and/or internal organs, most often the lungs (Certain categories of this form of scleroderma are more serious and can be fatal.)
Overlap syndrome—may involve features of scleroderma and features of other autoimmune syndromes
Overproduction of collagen and other connective tissue proteins is the main feature of scleroderma. It is not clear what causes this overproduction. Malfunction of the immune system may contribute to excess collagen production. There is also evidence that scleroderma may result from vascular abnormalities.
Symptoms are usually restricted to the skin. This disorder does not progress to involve internal organs. The skin lesions may completely reverse themselves in a few months or a few years. In some cases, they lead to permanent disfigurement. Symptoms include:
Hard patches on the skin, most often on the face or trunk (morphea)
Lines of thickened skin that can extend to underlying muscles and bones (linear scleroderma or linear morphea)
This form of the disease is typically categorized as either limited or diffuse disease. Many cases of limited disease begin gradually with
Raynaud's phenomenon. This involves swelling, tingling, numbness, blue and white color, and pain in fingers and toes. It is brought on by cold or emotional distress. The condition can progress over the years to thickened skin.
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