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. Scleroderma can run in families, but in most cases it occurs without any known family tendency for the disease. Scleroderma isn't considered contagious, but it can greatly affect self-esteem and the ability to accomplish everyday tasks.
Complications of Scleroderma
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.
Causes of Scleroderma
Signs & Symptoms of Scleroderma
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:
The two main varieties of localized scleroderma, which affects only the skin, are distinguished by very distinctive signs and symptoms:
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.
Diagnosis of Scleroderma
Your doctor may also conduct the following tests:
Your doctor may recommend other diagnostic tests to identify any lung, heart or gastrointestinal complications accompanying scleroderma.
Treatments of Scleroderma
Scleroderma has no known cure — no drug will stop the overproduction of collagen. But the localized variety of scleroderma sometimes resolves on its own. And a variety of medications can help control the symptoms of scleroderma or help prevent complications.