Scleroderma: causes, symptoms and treatment

Scleroderma, also known as systemic scleroderma, is a group of rare diseases that involve hardening and tightness of the skin. It can also cause problems with blood vessels, internal organs, and the digestive tract.

Scleroderma is often classified as limited or diffuse, referring to the degree of skin involvement. Both types can include any of the other vascular or organic problems. Localized scleroderma, also called morphea, only affects the skin.

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

Symptoms

The signs and symptoms of scleroderma vary from person to person depending on which parts of the body are affected.

Skin-related signs and symptoms

Almost all people with scleroderma have tough, tight skin.

Often the first parts of the body affected are the fingers, hands, feet and face. In some people, thickened skin may also appear on the forearms, upper arms, chest, abdomen, lower legs, and thighs. Early symptoms may include swelling and itching. Affected skin may become lighter or darker and may appear shiny due to stiffness.

Some people also have small red spots, called spider veins, on their hands and face. Calcium deposits can form under the skin, especially on the fingertips, causing lumps visible on x-rays.

Raynaud’s phenomenon

Raynaud’s phenomenon is common in scleroderma and is caused by inappropriate and excessive contraction of small blood vessels in the fingers and toes in response to cold or emotional distress. When this happens, your fingers may turn white, blue, or red and become numb or painful. Raynaud’s phenomenon can also occur in people who do not have scleroderma.

digestive problems

Scleroderma can affect any part of the digestive system, from the esophagus to the rectum. Depending on the parts of the body affected, signs and symptoms may include: heartburn difficulty swallowing abdominal bloating diarrhea constipation faecal incontinence

Heart and lung problems

When scleroderma affects the heart or lungs, it can cause shortness of breath, reduced tolerance for physical exertion, and dizziness. Scleroderma can cause scar tissue in the lungs, which can lead to increased shortness of breath over time. There are medications that can help slow the progression of lung damage.

Scleroderma can also cause increased blood pressure in the blood that travels 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.

Anyone can get scleroderma, but it is much more common in women than in men. Several combined factors seem to influence the risk of developing scleroderma: Genetic. People who have certain genetic variations seem to be more likely to develop scleroderma. This may explain why a small number of scleroderma cases appear to be hereditary and why certain types of scleroderma are more common in certain ethnic groups. Environmental triggers. Research suggests that, in some people, the symptoms of scleroderma can be triggered by exposure to certain viruses, medications, or illicit drugs. Repeated exposure, such as at work, to certain harmful substances or chemicals can also increase the risk of scleroderma. In most people, no environmental trigger is identified. Immune system problems. Scleroderma is considered an autoimmune disease. This means that it occurs partly because the body’s immune system begins to attack the connective tissues. People with scleroderma may also have symptoms of another autoimmune disease, such as rheumatoid arthritis, lupus, or Sjogren’s syndrome.

Diagnostic

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

After a complete physical exam, your doctor may suggest that you have blood tests to check for high levels of certain antibodies generated by the immune system.

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

Medications

Since scleroderma can affect many different parts of the body, the choice of medication will vary depending on the symptoms. Examples include drugs that can do the following: Dilate blood vessels. Blood pressure medications that dilate blood vessels can help treat Raynaud’s phenomenon. Suppresses the immune system. Drugs that suppress the immune system, such as those taken after an organ transplant, may help slow the progression of some symptoms of scleroderma, such as thickening of the skin or progression of lung damage. Reduce digestive symptoms. Stomach acid reducing tablets can help relieve heartburn. Antibiotics and medications that promote the movement of food through the intestines can help reduce bloating, diarrhea, and constipation. Prevent infections. Cleanliness and protection from the cold can help prevent infection of fingertip ulcers caused by Raynaud’s disease. Regular flu and pneumonia shots can help protect lungs damaged by scleroderma. Ease the pain. If over-the-counter pain relievers aren’t enough, your doctor may prescribe pain relievers.

Therapies

Physical therapists or occupational therapists can help you improve your strength and mobility and function independently in daily tasks. Manual therapy can help prevent hand contractures.

Surgeries and other procedures

Stem cell transplants could be an option for people who have severe symptoms and have not responded positively to most common treatments. If the lungs or kidneys have been severely damaged, organ transplants may be considered.