Scleroderma - Causes

Symptoms and Complications

Raynaud's Phenomenon

Raynaud's phenomenon is often the first sign of the scleroderma disease process. With this condition, small blood vessels constrict in the fingers, toes, ears, and sometimes even the nose.

Attacks of Raynaud's phenomenon can occur several times a day, and are often brought on or worsened by exposure to cold. Warmth relieves these attacks. In severe cases, attacks can develop regardless of the temperature. Severe cases may also cause open sores or damage to the skin and bones, if the circulation is cut off for too long.

Typically, the fingers go through three color changes:

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• First, they become very pale.
• As the blood flow is cut off, they turn a bluish color, usually in the top two sections of the second and third fingers.
• Finally, when blood flow returns, the fingers become red.

Tingling and pain can occur in the affected regions.

Click the icon to see an image of Raynaud's phenomenon.

Raynaud's is very common and occurs in 3 - 5% of the general population. It's important to note that more than 80% of patients with Raynaud's phenomenon do not have scleroderma, lupus, rheumatoid arthritis, or other serious illnesses. Raynaud's is more likely to be a symptom of scleroderma or some other connective tissue disease if it develops after age 30, if it is severe, and if it is accompanied by other symptoms (such as skin changes and arthritis).

Skin Changes

Course of Typical Skin Changes. The primary symptoms of scleroderma occur in the skin. They often take the following course:

• Typically, pitted scars appear first on the hands. The skin begins to thicken and harden on the hands, feet, and face. The fingers may swell. This condition is called sclerodactylia or acrosclerosis. Patients with diffuse scleroderma may have swelling of the whole hand before the skin significantly thickens.
• Thickened or hardened patches may also develop on other areas of the body. (Their appearance on the trunk and near the elbows or knees tends to be a sign of a more severe condition.)
• For the first 2 or 3 years, the skin continues to thicken and feel puffy.
• This process then stops, and can even get better. The skin may soften.
• As the disease progresses further, however, the skin loses its ability to stretch, and becomes shiny as it tightens across the underlying bone, particularly in the fingers, toes, and around the mouth.
• Eventually, in severe cases, the fingers may lose the ability to move, and can be difficult to bend. The hands and feet may curl from the tightness of the skin. It may be difficult to open the mouth widely.