Scleroderma is a chronic disease in which the skin and connective tissues become hardened and tightened by collagen deposits. It is usually localised, but can affect blood vessels and the whole body. This form may be fatal due to kidney, heart, lung and intestinal damage.
It is more common in adult women, and may run in families.
It is a form of auto-immune disease, in which a person’s antibodies are directed against his/her own tissues.
There is no known cause of scleroderma, though it is thought that the antibodies against tissues cause inflammation which then stimulates the production of excess collagen. This leads to hardening and eventually tightening of the affected tissues.
Symptoms will depend on whether the disease is limited to the skin, or whether it affects the whole body.
- swelling and redness of the skin, leading to
- thickened hardened skin – the face, hand and fingers are usually the first to be affected
- prutitus (itch) may be prominent
Other features are:
- Raynaud’s phenomenon: numbness and colour changes especially of the fingers and toes, triggered by cold exposure or emotions. This affects nearly all scleroderma patients
- stiffness of the joints
- digestive problems affect 90% of scleroderma patients- any part of the digestive tract can be involved, causing symptoms such as difficulty swallowing, diarrhoea, malabsorption
- lung problems occur in 70% of scleroderma patients – fibrosing alveolitis causes breathing problems, and pulmonary hypertension can be the result of the lung arteries being involved
- kidney damage leads to a decrease in function, and the development of hypertension in 60 – 80% of patients
- heart disease is common, with a five-year mortality rate of up to 75%. The heart may be directly involved (myocarditis, fibrosis), but heart problems may arise due to the hypertension and pulmonary hypertension.
- sexual dysfunction is common – impotence in men, and painful intercourse in women, due to vaginal dryness and constriction of the vagina.
- joint problems – true arthritis is rare, but joint and muscle pain is common.
- cancer: the incidence of lung cancer in scleroderma patients is five times higher than in the general population. The reason is unknown.
The diagnosis of scleroderma may not be obvious, as the symptoms overlap with many other disorders.
Each symptom must be investigated in its own right, for example:
- swallowing disorders, heart or lung problems
- blood tests may show a pattern of raised antibody levels in the blood, as found in auto-immune diseases, though this is not diagnostic
- A skin sample may show excess collagen formation
There is no known cure, and no known way to slow down collagen production.
Symptomatic treatment for dry skin is given, and topical steroids may reduce inflammation. Cosmetologists may help to disguise the redness on the face due to tiny blood vessels (telangiectasia), by using laser therapy, and skilfully applied make-up.
Hypertension and circulatory problems often can be treated successfully by
- calcium channel blockers
- ACE inhibitors
- enteric-coated low dose aspirin
Joint problems may be treated successfully with anti-inflammatories or steroids, and, in some cases, chemotherapy drugs such as methotrexate, and immune suppressants.
Intestinal problems are treated according to the problem presented.
Patients are advised to stay as active as possible, not to smoke (nicotine causes further spasm of the arteries), and protect themselves effectively from the cold. Support groups may be helpful.