Scleroderma (sklere-o-DUR-muh) is a group of rare, progressive diseases that involve the hardening and tightening of the skin and connective tissues — the fibers that provide the framework and support for your body.
Localized scleroderma affects only the skin. Systemic scleroderma also harms internal organs, such as the heart, lungs, kidneys and digestive tract. Scientists estimate that about 250 people per million have some form of scleroderma.
No one knows what causes this illness for sure. Genetics might play a role, but this is not for certain.
In the diffuse form of scleroderma, there is a thickening of the skin on the face, chest, flanks, and abdomen. This aggressive form goes on to make the skin hard after there has been an inflammatory period. The organ involvements usually happen early in this form and can attack the kidneys, heart, bowels, and lungs. It is not uncommon to have hypertension as well.
Scleroderma symptoms vary, depending on which organ systems are involved. Diagnosis can be difficult because some of the early symptoms are common in the general population, and aren't always associated with scleroderma.
The most prevalent signs and symptoms of scleroderma include:
- Raynaud's phenomenon - An exaggerated response to cold temperatures or emotional distress, this condition constricts the small blood vessels in the hands and feet and causes numbness, pain or color changes in the fingers or toes.
- Gastroesophageal reflux disease (GERD) - In addition to acid reflux, which can damage the section of esophagus nearest your stomach, you may also have problems absorbing nutrients if your intestinal muscles aren't moving food properly through your intestines.
- Skin changes - These changes may include swollen fingers and hands; thickened patches of skin, particularly on the fingers; and tight skin around the hands, face or mouth. Skin can appear shiny because it's so tight, and movement of the affected area may be restricted.
The two main varieties of localized scleroderma, which affects only the skin, are distinguished by very distinctive signs and symptoms:
- Morphea - This variety features oval-shaped thickened patches of skin that are white in the middle, with a purple border.
- Linear scleroderma - This variety, which is more common in children, features bands or streaks of hardened skin on one or both arms or legs, or on the forehead.
This type of scleroderma, also called systemic sclerosis, affects not only your skin but also your blood vessels and internal organs. Subcategories are defined by what portions of your body are affected. One variation is known as limited scleroderma, or CREST syndrome.
Scleroderma results from an overproduction and accumulation of collagen in body tissues. Collagen is a fibrous type of protein that makes up your body's connective tissues, including your skin.
Although doctors aren't sure what prompts this abnormal collagen production, the body's immune system appears to play a role. For unknown reasons, the immune system turns against the body, producing inflammation and the overproduction of collagen.
Several factors appear to increase the risk of certain types of scleroderma:
- Native Americans - Choctaw Native Americans in Oklahoma are at least 20 times more likely than is the general population to develop systemic scleroderma. Oddly enough, this increased risk doesn't apply to Choctaws living in Mississippi.
- African-Americans - Systemic scleroderma is more common in African-Americans than it is in Americans of European descent. And the African-Americans who have systemic scleroderma are more likely to develop severe lung complications.
Scleroderma occurs at least four times more often in women than it does in men.
Exposure to a variety of substances may be linked to the development of scleroderma.
- Silica dust, which is common in coal mines and rock quarries
- Some industrial solvents, such as paint thinners
- Certain chemotherapy drugs
Scleroderma complications can range from mild to severe. Some may even become life-threatening.
The variety of Raynaud's phenomenon that occurs with scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or ulcers in the flesh. In some cases, gangrene and amputation may follow.
Scarring of lung tissue (pulmonary fibrosis) can result in reduced lung function, reduced ability to breathe and reduced tolerance for exercise. You may also develop high blood pressure in the arteries to your lungs (pulmonary hypertension).
When scleroderma affects your kidneys, you can develop an elevated blood pressure and an increased level of protein in your urine. More serious effects of kidney complications may include renal crisis, which involves a sudden increase in blood pressure and rapid kidney failure.
Scarring of heart tissue increases your risk of abnormal heartbeats (arrhythmias) and congestive heart failure, and can cause inflammation of the membranous sac surrounding your heart (pericarditis).
Severe tightening of facial skin can cause your mouth to become smaller and narrower, which may make it hard to brush your teeth or to even have them professionally cleaned. People who have scleroderma often don't produce normal amounts of saliva, so the risk of decay increases even more. In addition, acid reflux can destroy tooth enamel, and changes in gum tissue may cause your teeth to become loose or even fall out.
Men who have scleroderma often experience erectile dysfunction. Scleroderma may also affect the sexual function of women, by decreasing sexual lubrication and constricting the vaginal opening.
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