What Is Scleroderma?
Scleroderma is an autoimmune disease that causes inflammation and fibrosis (thickening) in the skin and other areas of the body. When an immune response tricks tissues into thinking they are injured, it causes inflammation, and the body makes too much collagen, leading to scleroderma. Too much collagen in your skin and other tissues causes areas of tight, hard skin. Scleroderma may involve many systems in your body.
There are two major types of scleroderma:
- Localized scleroderma only affects the skin and the structures directly under the skin.
- Systemic scleroderma, also called systemic sclerosis, affects many systems in the body. This is the more serious type of scleroderma and can damage your blood vessels and internal organs, such as the heart, lungs, and kidneys. This subset is also divided into two additional categories called "limited" and "diffuse" which represents how much skin involvement there is in the body.
There is no cure for scleroderma. The goal of treatment is to relieve symptoms and stop the progression of the disease. Early diagnosis and ongoing monitoring are important.
What happens in scleroderma?
The cause of scleroderma is unknown. However, researchers think that the immune system overreacts and causes inflammation and injury to the cells that line blood vessels. This triggers connective tissue cells, especially a cell type called fibroblasts, to make too much collagen and other proteins. The fibroblasts live longer than normal, causing a buildup of collagen in the skin and other organs, leading to some of the signs and symptoms of scleroderma. There can also be injuries to blood vessels.
Source: National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)