What is Scleroderma?
Scleroderma is the chronic hardening and tightening of the skin and connective tissues. (Also called Crest Syndrome)
- Fewer than 200,000 US cases per year
- Treatment can help, but this condition can’t be cured
- Requires a medical diagnosis
- Lab tests or imaging always required
- Chronic: can last for years or be lifelong
- Scleroderma is a group of rare diseases that more often affects women. It commonly occurs between the ages of 30 and 50.
- Symptoms include tightening of the skin, joint pain, exaggerated response to cold (Raynaud’s disease), and heartburn.
- Treatments include medication, physical therapy, and surgery.
A Closer Look
Scleroderma’s signs and symptoms vary, depending on which parts of your body are involved:
- Nearly everyone who has scleroderma experiences a hardening and tightening of patches of skin. These patches may be shaped like ovals or straight lines, or cover wide areas of the trunk and limbs. The number, location and size of the patches vary by type of scleroderma. Skin can appear shiny because it’s so tight, and movement of the affected area may be restricted.
- Fingers or toes.One of the earliest signs of scleroderma is an exaggerated response to cold temperatures or emotional distress, which can cause numbness, pain or color changes in the fingers or toes. Called Raynaud’s disease, this condition also occurs in people who don’t have scleroderma.
- Digestive system.In addition to acid reflux, which can damage the section of esophagus nearest the stomach, some people with scleroderma may also have problems absorbing nutrients if their intestinal muscles aren’t moving food properly through the intestines.
- Heart, lungs or kidneys.Scleroderma can affect the function of the heart, lungs or kidneys to varying degrees. These problems, if left untreated, can become life-threatening.
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.
Doctors aren’t certain what prompts this abnormal collagen production, but the body’s immune system appears to play a role. In some genetically susceptible people, symptoms may be triggered by exposure to certain types of pesticides, epoxy resins or solvents.
Scleroderma occurs more often in women than it does in men.
Scleroderma complications range from mild to severe.
- The variety of Raynaud’s disease that occurs with scleroderma can be so severe that the restricted blood flow permanently damages the tissue at the fingertips, causing pits or skin sores (ulcers). 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 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). Scleroderma can also raise the pressure on the right side of your heart and cause it to wear out.
- 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 dental decay increases even more.
- Digestive problems associated with scleroderma can lead to acid reflux and difficulty swallowing — some describe feeling as if food gets stuck midway down the esophagus — as well as bouts of constipation alternating with episodes of diarrhea.
- 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.
How is Scleroderma diagnosed?
After a thorough physical exam, your doctor may suggest blood tests to check for elevated blood levels of certain antibodies produced by the immune system. He or she may remove a small tissue sample (biopsy) of your affected skin so that it can be examined in the laboratory for abnormalities.
No drug has been developed that can stop the underlying process of scleroderma — the overproduction of collagen. But a variety of medications can help control scleroderma symptoms or help prevent complications.