Scleroderma.

Last updated Tuesday, December 28, 2004

Figure 1 - Types of scleroderma

Symptoms

Symptoms

Scleroderma affects everyone differently. It can be mild in some people, and more severe in others. Each form of scleroderma affects the body in different ways. Below are some of the ways the disease may affect the body.

Types of scleroderma

There are several different forms of scleroderma (see figure 1). Localized scleroderma affects mainly the skin. It can affect skin in different areas of the body. It may also affect muscles and bone, but it does not affect internal organs. This form is usually not as severe as generalized scleroderma. People who develop localized scleroderma usually do not develop generalized scleroderma.

Morphea scleroderma happens when hard, oval-shape patches form on the skin. The patches are usually whitish with a purplish ring around them. They usually occur on the trunk, but can also occur on the face, arms, legs, and other parts of the body. Morphea often improves by itself, over time.

Linear scleroderma is a line of thickened skin that occurs in areas such as the arms, legs, or forehead. It can occur in more than one area. The line can extend deep into the skin and affect the bones and muscles underneath it. This can affect the motion of joints and muscles, as well as the growth of the affected area. When the line forms a long crease on the head or neck, it is sometimes called en coup de sabre--the strike of a sword. Linear scleroderma usually occurs in childhood.

Generalized scleroderma affects many parts of the body. It can affect the skin as well as internal body parts, such as blood vessels, the digestive system (esophagus, stomach, and bowel), the heart, lungs, kidneys, muscles, and joints. The severity of this form depends on the organs affected and how much they are affected. In rare cases, scleroderma may affect only some internal system, leaving the skin and joints untouched.

There are two types of generalized scleroderma: limited (also called the CREST syndrome) and diffuse.

• CREST stands for a combination of symptoms: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia. This type, also known as limited scleroderma, usually has a slow onset, with the first symptoms appearing 10 to 20 years before the full syndrome occurs. It usually affects the skin on the face, fingers and hands. Later on, it may affect internal organs, such as the esophagus (the tube leading from mouth to the stomach), the lungs and bowels.
• Diffuse occurs throughout the body. It usually affects the skin as well as other body parts, such as the lungs, kidneys, heart, bowels, blood vessels and joints. Depending on the areas affected, this type can cause problems such as high blood pressure, muscle weakness, trouble swallowing, or shortness of breath. Diffuse scleroderma may progress slowly in some people and more rapidly in others. However, with proper management, it can usually be controlled.

Skin changes

Skin changes occur in most people with scleroderma. These changes include:

• hardening and thickening of skin, especially on the hands, arms, and face
• ulcers on the fingers
• decrease in hair over the affected area
• change in skin color

Swelling

Swelling or puffiness of the hands and feet is another common symptom of scleroderma. This often happens in the morning.

You may notice:

• skin on fingers and toes may look and feel swollen
• shiny skin
• usual skin creases disappear
• tight skin--it may be difficult to make a fist
• numbness and tingling in fingers

Sclerodactyly

Sclerodactyly means "scleroderma of the digits" (fingers and toes). It usually occurs after the initial swelling goes away.

You may notice:

• skin on fingers and toes becomes hard and shiny
• difficulty bending fingers
• contractures in fingers

Raunaud's phenomenon

Raynaud's phenomenon is a problem of poor blood flow to fingers and toes. Blood flow decreases because blood vessels in these areas become narrow for a short time, in response to cold or to emotional stress. Sometimes all it takes to trigger this reaction is walking into a cold room or reaching into the refrigerator. This is an early and common symptom of scleroderma.

You may notice:

• fingers, toes, and sometimes the tips of the ears, nose, or tongue, are very sensitive to cold
• fingers turn bluish or very pale
• tingling, numbness or a cold sensation in the fingers

When the hands warm up, the blood vessels open and the skin color returns to normal as the blood supply to the fingers improves.

A note about smoking: If you smoke, stop. In addition to all the other health reasons for not smoking, smoking can trigger attacks of Raynaud's phenomenon. Contact the American Cancer Society, American Heart Association, American Lung Association, or your doctor to learn about ways to quit smoking.

Telangiectasia

Telangiectasia happens when tiny blood vessels near the surface of the skin show through the skin.

You may notice:

• small reddish spots on fingers, palms, face, lips, and/or tongue

The spots are not harmful and can be hidden with cosmetics.

Calcinosis

Calcinosis happens when small white calcium lumps form under the skin. This is due to scleroderma, and is NOT caused by too much calcium in your diet.

You may notice hard white lumps under the skin on fingers or other areas of the body. The lumps may break through the skin and leak a chalky white liquid.

Arthritis and muscle weakness

Arthritis and muscle weakness may also be symptoms of scleroderma. Arthritis happens when joints become painful and swollen. A contracture happens when the skin and tissues around a joint become tight and hard causing the joint to tighten into a bent position. This can happen in the hands and other parts of the body.

You may notice:

• pain, stiffness, swelling, warmth and tenderness in joints
• general fatigue
• weak muscles, often in shoulder or hip

Sjogren's syndrome

Sjogren's syndrome is a decrease in secretions from the tear ducts, salivary glands and other areas of the body, such as the vagina. This happens if scleroderma affects the glands that produce these fluids.

You may notice:

• dry eyes and mouth
• trouble speaking or swallowing food
• dryness in the vagina or other areas of the body
• increase in dental cavities

Digestive problems

The digestive system includes the mouth, esophagus, stomach, and bowels. Scleroderma can weaken the esophagus and the bowels. It can also cause a build-up of scar tissue in the esophagus, which narrows the tube.

You may notice:

• difficulty swallowing
• heartburn
• bloating
• nausea or vomiting
• weight loss
• diarrhea or constipation

Heart and lung problems

Scleroderma may cause the heart to slow down and, in some cases, can lead to heart failure or other problems. When the lungs are affected, they cannot function as well.

You may notice:

• shortness of breath
• a persistent cough
• chest pain

Kidney problems

Scleroderma can cause high blood pressure and kidney failure. If not treated, this can be a serious problem. You should be aware of the signs of kidney problems, listed here.

You may notice:

• headache
• shortness of breath
• visual disturbances
• chest pain
• mental confusion

If you notice any of these signs, call your doctor right away so the problem can be treated.

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