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Scleroderma

Medically reviewed by Drugs.com. Last updated on Jan 17, 2024.

Overview

Scleroderma (sklair-oh-DUR-muh), also known as systemic sclerosis, is a group of rare diseases that involve the hardening and tightening of the skin. It may also cause problems in the blood vessels, internal organs and digestive tract.

Scleroderma is often categorized as "limited" or "diffuse," which refers only to the degree of skin involvement. Both types can involve any of the other vascular or organ problems. Localized scleroderma, also known as morphea, affects only the skin.

While there is no cure for scleroderma, treatments can ease symptoms, slow progression and improve quality of life.

Symptoms

Scleroderma's signs and symptoms vary from person to person, depending on which parts of the body are affected.

Skin-related signs and symptoms

Nearly everyone who has scleroderma experiences a 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 can also involve the forearms, upper arms, chest, abdomen, lower legs and thighs. Early symptoms may include swelling and itchiness. Affected skin can become lighter or darker in color and may look shiny because of the tightness.

Some people also experience 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.

Raynaud's phenomenon

Raynaud's phenomenon is common in scleroderma and occurs because of an inappropriate and exaggerated contraction of the small blood vessels in the fingers and toes in response to the cold or emotional distress. When this happens, the digits may turn white, blue or red, and feel painful or numb. Raynaud's phenomenon also can occur in people who don't have scleroderma.

Digestive problems

Scleroderma can affect any part of the digestive system, from the esophagus to the rectum. Depending on which parts of the digestive system are affected, signs and symptoms may include:

Heart and lung problems

When scleroderma affects the heart or lungs, it can cause shortness of breath, decreased exercise tolerance and dizziness. Scleroderma can cause scarring in the lung tissues that may result in increasing shortness of breath over time. There are medications that may help slow the progression of this lung damage.

Scleroderma can also cause the blood pressure to increase in the circulation that goes between the heart and the lungs. This is called pulmonary hypertension. In addition to shortness of breath, pulmonary hypertension can also cause excess fluid in the legs, feet and sometimes around the heart.

When scleroderma affects the heart, heartbeats can become irregular. Heart failure may also occur in some people.

Causes

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 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 immune system problems, genetics and environmental triggers.

Risk factors

Anyone can get scleroderma, but it does occur much more often in women than in men. Several combined factors appear to influence the risk of developing scleroderma:

Complications

Scleroderma complications range from mild to severe and can affect the:

Diagnosis

Because scleroderma can take so many forms and affect so many different areas of the body, it can be difficult to diagnose.

After a thorough physical exam, your doctor may suggest blood tests to check for elevated levels of certain antibodies produced by the immune system.

Your doctor may also suggest other blood tests, imaging or organ-function tests to help determine whether your digestive system, heart, lungs or kidneys are affected.

Treatment

There is no treatment that can cure or stop the overproduction of collagen that is characteristic of scleroderma. But a variety of treatments can help control symptoms and prevent complications.

Medications

Because scleroderma can affect so many different parts of the body, the choice of medication will vary, depending on the symptoms. Examples include drugs that:

Therapies

Physical or occupational therapists can help you improve your strength and mobility and maintain independence with daily tasks. Hand therapy may help prevent hand contractures.

Surgical and other procedures

Stem cell transplants might be an option for people who have severe symptoms that haven't responded to more-common treatments. If the lungs or kidneys have been severely damaged, organ transplants might be considered.

Lifestyle and home remedies

You can take a number of steps to help manage your symptoms of scleroderma:

Coping and support

As is true with other chronic diseases, living with scleroderma can place you on a roller coaster of emotions. Here are some suggestions to help you even out the ups and downs:

Keep in mind that your physical health can have a direct impact on your mental health. Denial, anger and frustration are common with chronic illnesses.

At times, you may need additional tools to deal with your emotions. Professionals, such as therapists or behavior psychologists, may be able to help you put things in perspective. They can also help you develop coping skills, including relaxation techniques.

Joining a support group, where you can share experiences and feelings with other people, is often a good approach. Ask your doctor what support groups are available in your community.

Preparing for an appointment

You'll probably first bring your symptoms to the attention of your family doctor, who may refer you to a doctor who specializes in the treatment of arthritis and other diseases of the joints, muscles and bone (rheumatologist). Because scleroderma can affect many organ systems, you may need to see a variety of medical specialists.

What you can do

Time with your doctors may be brief. To make the best use of the limited time, plan ahead and write lists of important information, including:

What to expect from your doctor

Your doctor is likely to ask questions, such as:

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