Scleroderma is a group of diseases that causes hardening and tightening of the skin and connective tissues. Scleroderma is rare, affecting an estimated 250 people per million, according to the MayoClinic.com. Localized scleroderma often affects only the skin while systemic scleroderma can damage the internal organs, such as the heart, lungs, digestive tract and kidneys. The development of scleroderma is the result of a complex immune response.
Causes
Scleroderma appears to occur due to an autoimmune response, in which the immune system begins to attack itself. In scleroderma, the response produces inflammation and too much production of collagen, according to the University of Maryland Medical Center. Collagen is a tough protein that helps build connective tissues such as tendons, ligaments and bones as well as scar tissue. Scleroderma results when an overproduction and accumulation of collagen settles in the tissues of the body.
Risk Factors
Certain people seem to have a greater risk of developing scleroderma. Native Americans, especially the Choctaw tribe in Oklahoma, are 20 times more likely than the rest of the population to develop systemic scleroderma, according to the MayoClinic.com. African-Americans also have a greater likelihood of developing systemic scleroderma with severe lung complications than Americans of European descent. The University of Maryland Medical Center explains that the incidence of scleroderma is three to eight times greater in women than men.
Symptoms
When scleroderma affects only the skin, the symptoms often include skin discoloration such as blueness or redness of fingers and toes in response to heat and cold, skin hardness, thickened skin, stiffness and tightness as well as small white lumps under the skin that oozes a substance that looks like toothpaste, according to the National Institutes of Health. When scleroderma is systemic, symptoms vary depending on where they occur. These symptoms can include joint pain, numbness in the feet, dry cough, shortness of breath, wheezing, bloating after meals, constipation, diarrhea and difficulty swallowing.
Diagnosis
No specific test can identify scleroderma. Diagnosis of scleroderma commonly involves a thorough examination of the skin to look for thickening and hardening, crackling or grating sensations within the joints and microscopic changes in the tissues of the fingernails. The University of Maryland Medical Center explains that physicians also commonly run blood tests to look for particular substances that help identify scleroderma.
Treatment
Medications can be used to help dilate the blood vessels to help prevent complications in the lungs and kidneys. Physicians may also prescribe medications to help suppress the immune response. Some patients undergo cosmetic procedures to help with skin lesions. In some cases, amputation may be necessary if a lack of blood flow to the fingers has resulted in gangrene, according to the MayoClinic.com.
