There are important concepts to understand about scleroderma:
- No two patients are exactly the same and the clinical expression of scleroderma varies a great deal among patients.
- Most patients have mild disease with annoying but not life-threatening disease.
- Scleroderma is treatable, resulting in a long and excellent quality of life.
- The exact management plan must be customized to meet the individual patient’s needs.
- Scleroderma can be severe with systemic organ disease and treatment needs to start early to prevent complications.
- The disease process can go into a natural remission and features not progress or even improve.
- There are many ways to CLASSIFY patients to define “TYPE”. Classification of a patient helps define treatment and predict the future course of scleroderma.
- The most popular classification is the degree of skin changes (scleroderma means hard skin). Using the skin classification there are two groups: Limited and Diffuse skin involvement (see below).
- We now appreciate that the simple two group classification does not fully capture the complexity and variation in the disease course that scleroderma can follow. Now there are many more important and precise ways to classify patients. These include modern methods of looking at the whole patient and not just the skin including internal organs, special laboratory and biological measures. Some methods of classification include:
a. A composite score including the degree of skin, level of other organ systems and biomarkers
b. Stratify by autoantibodies (blood test measuring the immune response)
c. Molecular genetic Profiling
d. Novel Biomarkers in the skin or blood
- The MOST important message is that while defining the features (type) helps guide the doctor; the actual patient’s experience as expressed by bedside exam, special testing and serial revisits overtime is the key to ideal management.
The classification of scleroderma by skin manifestations:
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.