There are two main types of systemic sclerosis: limited cutaneous systemic sclerosis and diffuse cutaneous systemic sclerosis.
If an individual has limited cutaneous systemic sclerosis, the skin involvement is limited to the fingers, hands, lower arms, lower legs and face. This type of systemic sclerosis was previously referred to as CREST syndrome [which stands for calcinosis, Raynaud's symptoms, (o)esophageal problems, sclerodactyly and telangiectasia]. However, we now know that these problems also occur in individuals with diffuse systemic sclerosis as well and we therefore avoid the term "CREST".
If an individual has diffuse cutaneous systemic sclerosis, the skin involvement extends beyond the fingers, hands, forearms, lower legs and face, to also include the chest wall or other areas of the body.
The diagram below shows in purple the areas of skin that are involved in limited systemic sclerosis (on the left) and diffuse systemic sclerosis (on the right).
Problems people with systemic sclerosis may encounter include:
- Tightening of the skin around the fingers, the face and other areas of the body causing contractures (fixed joints);
- Raynaud's phenomenon: where the small blood vessels of the fingers undergo spasm, triggered by the cold, a change in temperature, or a stressful event. Other exposed areas of the skin such as the toes, ears, and nose may be affected;
- Skin ulceration, dryness and irritation;
- Reduced mouth opening (microstomia) which can cause difficulty eating and make dental hygiene hard to maintain;
- Broken blood vessels (telangiectasia) on the face and hands;
- Calcinosis (small deposits of calcium developing under the skin) which can be painful, and cause ulcers and infections;
- Gut problems, from heartburn and difficulty swallowing, to constipation and/or diarrhoea;
- In some patients, lung problems including scarring or pulmonary hypertension (abnormally high blood pressure in the arteries of the lungs) and kidney problems with hypertension may develop.
There are various medications that can be used to treat the different symptoms. In addition to any medications, physiotherapy and hand exercises are important. Warm paraffin wax baths can also be useful for people with problematic skin and moisturising the skin is essential. Our booklet on physiotherapy in scleroderma (pdf) is a useful source of exercises that can be tried.
The majority of people with systemic sclerosis lead full and productive lives, though everyone should be closely followed up and have annual tests for organ problems as the earlier problems are detected, the more effectively they can be treated.