July 3, 2024 | It’s estimated that about 300,000 Americans have scleroderma, an autoimmune disease that causes inflammation and thickening of the skin and other parts of the body. The term comes from the Greek word sclero, meaning hard, and the Latin word derma, meaning skin. The two main types of scleroderma are localized scleroderma, which only affects your skin and the muscles and tissues just under your skin, and systemic scleroderma, which is affects many parts of the body and can damage your blood vessels and internal organs, such as your heart, lungs, and kidneys. About one third of those people with the disease have the systemic form. Since scleroderma presents with symptoms similar to other autoimmune diseases, diagnosis can be difficult. Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults. Overall, females with scleroderma outnumber males about four to one.

July 3, 2024 | The autoimmune disease scleroderma happens when the body produces too much collagen and it builds up in body tissues. Collagen is a fibrous type of protein that makes up the body’s connective tissues, including the skin. Experts don’t know exactly what causes this process to begin, but the body’s immune system appears to play a role. Most likely, scleroderma is caused by a combination of factors, including genetics and environmental triggers. Research suggests that in some people, scleroderma symptoms may be triggered by exposure to certain viruses or drugs. Repeated exposure, such as at work, to harmful substances or chemicals also may increase the risk. People who have certain gene changes appear to be more likely to develop the disease. This may explain why scleroderma appears to run in families in a small number of people and why some types of scleroderma are more common for people in certain racial and ethnic groups.

July 3, 2024 | The symptoms of scleroderma can vary from person to person, depending on which parts of the body are affected. Nearly everyone who has the autoimmune disorder experiences hardening and tightening of the skin. The first parts of the body to be affected are usually the fingers, hands, feet and face. In some people, the skin thickening also can involve the forearms, upper arms, chest, abdomen, lower legs and thighs. The color of affected skin can become lighter or darker, and skin may look shiny because of the tightness. Some people also have small red spots, called telangiectasia, on their hands and face. Calcium deposits can form under the skin, particularly at the fingertips, causing bumps that can be seen on X-rays. An early, common sign of scleroderma is color changes to the fingers called Raynaud’s phenomenon. Fingers or toes may look red, white, or blue, especially in cold temperatures and may be swollen, numb or develop ulcers.

July 3, 2024 | When scleroderma affects the heart or lungs, it can cause shortness of breath, decreased exercise tolerance and dizziness. There are medicines that may help slow the progression of this lung damage. Scleroderma can also cause a person’s blood pressure to increase in the circulation that goes between the heart and the lungs. This is called pulmonary hypertension. In addition to causing shortness of breath, pulmonary hypertension also can cause excess fluid to build up in the legs, feet and sometimes around the heart. When scleroderma affects the heart, heartbeats can become irregular. Heart failure also may happen in some people. Digestive complications of the disease can include heartburn and difficulty swallowing. Scleroderma also can cause bouts of cramps, bloating, constipation or diarrhea. Some people who have scleroderma may also have problems absorbing nutrients due to the overgrowth of bacteria in the intestine.

July 3, 2024 | Because there is no cure for the autoimmune disease scleroderma, most treatments focus on controlling the patient’s symptoms. Certain medications can be prescribed to help decrease the swelling, manage the pain and prevent any complications from the disease. Physical or occupational therapy may help with pain, improve the patient’s muscle strength and teach them ways to help with daily living. Regular dental care is another option, because scleroderma can make your mouth dry and damage connective tissues in your mouth. These problems can speed up tooth decay and cause your teeth to become loose. Many people with scleroderma will see a rheumatologist. This is a doctor who specializes in rheumatic diseases such as arthritis and other inflammatory or autoimmune disorders. Dermatologists, who specialize in conditions of the skin, hair, and nails, may also play a significant role in treating the disease.