Scleroderma is an activation of immune cells, which produces scar tissue in the skin, internal organs, and small blood vessels. Women are three times more likely than men to develop the condition. Although scleroderma appears to be more common among African American women than other races, a woman's risk of developing scleroderma is 15 times greater during her childbearing years.
The two types of scleroderma are:
Localized scleroderma affects only certain parts of the body, while systemic scleroderma can affect the whole body.
Each type of scleroderma has subtypes. One of the subtypes of systemic scleroderma is limited scleroderma, which doctors refer to as CREST syndrome.
(Click Types of Scleroderma for more information on the different types.)
People with systemic scleroderma often have all or some of the symptoms that doctors refer to as CREST. CREST stands for the following:
CREST and Limited Scleroderma
Some doctors refer to limited
scleroderma as CREST syndrome because of the predominance of CREST symptoms.
Limited scleroderma typically comes on gradually and affects the skin only in certain areas, which include:
- Fingers
- Hands
- Face
- Lower arms
- Legs.
Many people with CREST syndrome have
Raynaud's phenomenon for years before skin thickening starts. Other people with CREST syndrome start out with skin problems over much of the body, which can improve over time, leaving only the face and hands with tight, thickened skin. In CREST syndrome, telangiectasias and calcinosis often follow.
As mentioned previously, symptoms of CREST include:
- Calcinosis. The formation of calcium deposits in the connective tissues, which can be detected by an x-ray. They are typically found on the fingers, hands, face, and trunk and on the skin above the elbows and knees. Painful ulcers may appear if the deposits break through the skin.
- Raynaud's phenomenon. A condition in which the small blood vessels of the hands and/or feet contract in response to cold or anxiety. As the vessels contract, the hands or feet turn white and cold, then blue. As blood flow returns, they become red. Fingertip tissues may suffer damage, leading to ulcers, scars, or gangrene.
- Esophageal dysfunction. Impaired function of the esophagus (the tube connecting the throat and the stomach) that occurs when the smooth muscles in the esophagus lose normal movement. In the upper esophagus, the result can be swallowing difficulties; in the lower esophagus, the problem can cause chronic heartburn or inflammation.
- Sclerodactyly. Thick and tight skin on the fingers resulting from deposits of excess collagen within skin layers. This condition makes it harder to bend or straighten the fingers, and may also cause skin to appear shiny and darkened, with hair loss.
- Telangiectasias. Small red spots on the hands and face that are caused by the swelling of tiny blood vessels. While these spots are not painful, they can create cosmetic problems.
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