Types of Scleroderma
Learn more about the different types of scleroderma according to the Johns Hopkins Scleroderma Center.
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.
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.