Scleroderma
What is scleroderma?
Scleroderma is a disorder of the immune system that causes thickening and hardening of the connective tissue in the skin and organs of the body. The exact cause remains unknown. Scleroderma is not contagious, inherited or passed down in families. It occurs more frequently in women than in men.
Scleroderma can be either localized (affects certain parts of the body) or systemic (affects the whole body). The two types of localized scleroderma are morphea and linear scleroderma (see DermNote on morphea for further information), and the two types of systemic scleroderma are limited and diffuse scleroderma.
Individuals with limited systemic scleroderma (formerly known as CREST syndrome) typically experience a set of symptoms which include: Raynaud’s phenomenon (described below), tightness of the skin of the fingers or toes, dysfunction of the esophagus, the appearance of calcium deposits, and the presence of small dilated blood vessels in the skin.
Individuals with diffuse systemic scleroderma experience hardening of the skin, tendons, blood vessels and internal organs (including the heart, lungs and bowels). This results in a wide range of symptoms and complications.
What are the symptoms of scleroderma?
Symptoms of scleroderma vary from person to person. They can range from very mild to life-threatening, depending on the area of the body affected and severity of the disease. In some cases, the symptoms seen in diffuse systemic scleroderma may also sometimes be seen in limited scleroderma, and vice-versa.
Symptoms of scleroderma in the skin include stiffness and tightness of the skin overlying the fingers, hands and/or forearms. This process can also affect the face, causing the skin to feel tight and mask-like. Skin affected by scleroderma can change color and become either darker or lighter. Hair loss of the affected skin is also common among individuals with scleroderma. Intense itching of the skin may also develop during the early stages of the disease. In some individuals with scleroderma, calcium deposits may form under the skin. These appear as small lumps which can ooze and, in some cases, form ulcers. These calcium deposits can occur elsewhere in the body, including the tendons and joints, and cause pain, numbness or stiffness.
The blood vessels in the body can become smaller, reduced in number and less able to function to provide blood to the tissues. As a consequence, fingers and/or toes may turn blue or white in response to either hot or cold temperature and become severely painful. This is known as Raynaud’s phenomenon.
The lungs may be involved, causing cough and difficulty breathing. Involvement of the gastrointestinal tract may cause difficulty swallowing, constipation, diarrhea or abdominal pain.
Generalized symptoms including weakness, fatigue, pain and sleeping problems may also be present. Because of the wide range of symptoms, diagnosing scleroderma is not easy and may take some time.
How is scleroderma treated?
Currently, there is no cure for scleroderma. However, strategies are available to help manage the effects of scleroderma and prevent further damage. Many of the management strategies for scleroderma are directed at the particular symptoms.
Patients who develop stiff joints should engage in frequent stretching exercises under the guidance of a physical or occupational therapist. This will help to maintain strength, flexibility and range of motion in the affected joints. For patients suffering from muscle and/or joint pain, anti-inflammatory drugs and pain-relieving medications may be prescribed.
Patients with Raynaud’s phenomenon should avoid temperature extremes. Proper dress should be worn, with special attention paid to the hands and feet. For extreme cases of Raynaud’s, including those involving the formation of sores or ulcers, your doctor may prescribe an oral medication. Smoking damages blood vessels and should be avoided.
In general, treatment for scleroderma is dependent on the severity of the disease and the organ systems involved. Because scleroderma can affect several body systems, you may also have other doctors to monitor the joints, kidneys, lungs, gastrointestinal tract, mouth and eyes.
What can I expect from treatment?
Not all treatments for scleroderma work equally well. There is a great deal of variability in the response to treatment among people with scleroderma. You may have to work with your doctors to find the best treatment for your specific set of symptoms.
When should I be concerned/contact my physician?
Seeing your doctor as soon as you first notice symptoms is an important step in treating scleroderma. This is because scleroderma is more difficult to treat if it is allowed to progress.
Scleroderma may be a difficult disorder to live with both socially and psychologically. You should talk to your doctor if you are having difficulty dealing with the effects of scleroderma as they may be able to help guide you towards ways to cope.