Medical Health Encyclopedia

Limited Scleroderma or CREST Syndrome. Limited scleroderma, also called CREST, is progressive and unremitting. It is classified as a systemic because its effects can be widespread. It generally differs from diffuse scleroderma in the following way:

• Most often the internal organs are not affected.
• It has a positive outlook unless pulmonary hypertension develops (a particular danger with the CREST syndrome).

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Limited scleroderma is commonly referred to by the acronym CREST, whose letters are the first initials of characteristics that are typically found in this syndrome:

• Calcinosis. With this condition, mineral crystal deposits form under the skin usually around the joints. Skin ulcers filled with a thick white substance may form over the deposits.
• Raynaud's phenomenon. In this syndrome, the fingers of both hands are very sensitive to cold, and they remain cold and blue colored after exposure to low temperatures. This occurs in nearly all cases of scleroderma, both limited and diffuse. It is caused by abnormal changes in the small arteries and capillaries, which result in constriction and a temporary interruption of circulation, usually in the fingers.
• Esophageal motility dysfunction. When this occurs, the muscles in the esophagus become scarred by scleroderma and do not contract normally. This can cause severe heartburn and other symptoms of gastroesophageal reflux disorder (GERD).
• Sclerodactylia (also called acrosclerosis). This is the stiffness and tightening of the skin of the fingers, a classic symptom of scleroderma. Bone loss may occur in the fingers and toes.
• Telangiectasia. In this situation, dilation of small vessels and capillaries cause numerous flat red marks on the hands, face, and tongue.

Click the icon to see an image of symptoms that are known as CREST.