Limited Scleroderma – CREST Syndrome
Limited scleroderma means only limited areas of skin are thick; usually just the fingers and/or face. Limited scleroderma is the milder form of scleroderma. It is more common among Caucasians than other populations. Every person with scleroderma is different and has a different pattern of symptoms. The CREST syndrome is a type of limited scleroderma. CREST stands for the following:
|C||(calcinosis) is for the calcium deposits under the skin and in tissues.|
|R||stands for Raynaud’s phenomenon.|
|E||is for esophageal dysmotility. This causes heartburn, which is often experienced by CREST patients.|
|S||is for sclerodactyly; that means thick skin on the fingers.|
|T||is for telangiectasias, which are enlarged blood vessels. These appear as red spots on the face and other areas.|
Calcium in the skin and tissues can be a painful annoyance that can irritate or break the skin surface. Raynaud’s attacks can be uncomfortable and associated with cold intolerance. Acid reflux from esophageal dysmotility can be painful causing irritation in the lining of the esophagus. The skin changes in limited scleroderma usually do not progress. Telangiectasia are not harmful and can be removed by laser therapy. Generally, limited scleroderma causes less involvement of body organs than the more severe form. Some patients can develop lung and heart disease. The doctors at The Johns Hopkins Scleroderma Center offer treatment options that can provide patients relief and prevent complications. Further information about treatments is available elsewhere on this website.
A second type of scleroderma is called diffuse scleroderma. This means that more areas of the skin are involved and thickened, but there is a high degree of variability among patients. Skin of the arms, legs, and trunk are more likely to be involved. The tightened skin makes it difficult to bend fingers, hands, and other joints. There is sometimes inflammation of the joints, tendons and muscles. Tight skin on the face can reduce the size of a person’s mouth and make good dental care very important.
The skin can lose or gain pigment; making areas of light or dark skin. Some people lose hair on the limbs, sweat less, and develop dry skin because of skin damage. Information about skin care can be found in the section of this website on living with scleroderma.
Diffuse scleroderma can have associated involvement of internal organs such as the gastrointestinal tract, heart, lungs, or kidneys. The degree of organ involvement is highly variable – some get none at all and other patients organs may be badly affected. Treatments for organ involvement in scleroderma are covered in the section on treatment options.