VOL: 101, ISSUE: 49, PAGE NO: 21WHAT IS IT?
WHAT IS IT?
- Scleroderma literally means hard skin.
- Scleroderma is an umbrella term for a number of conditions.
- Increased collagen is laid down in the lower dermis of the skin as well as the internal organs.
- The peak incidence is between 30 and 50 years of age.
- Limited cutaneous systemic sclerosis usually starts with Raynaud's phenomenon.
- Tightness of the skin over the fingers leads to flexion deformities and involvement of facial skin produces a beak-like nose and small mouth.
- Painful ulcers and occasionally gangrene of the digits occur. Gastrointestinal involvement is common.
- The lungs' involvement can lead to pulmonary fibrosis and, in 15 per cent of patients, pulmonary hypertension occurs later with heart failure and premature death.
- This form of scleroderma is known as CREST syndrome (Calcinosis, Raynaud's, Eosophageal involvement, Sclerodactyly and Telangiectasia).
DIFFUSE CUTANEOUS SYSTEMIC SCLEROSIS
- This is responsible for about 40 per cent of cases and has no universal cure.
- The skin is initially oedematous followed by rapid sclerosis, which can involve the whole body.
- Raynaud's phenomenon often occurs at the same time.
- There is early involvement of internal organs. Ten per cent of patients develop renal dysfunction with renal failure, which requires rapid treatment if recovery is to be achieved.
- Pulmonary involvement with fibrosis and pulmonary hypertension occurs.
- Oesophageal involvement and small bowel problems, pericarditis and pericardial effusion feature.
- It is important to diagnose and stage the disease.
- Treatment is targeted towards the organ involved.
- This form of scleroderma is confined to the skin and does not affect the internal organs.
- It is more common in children.
- There are two main types: morphoea and linear.
- Patches of thickened skin, which may be rounded or oval, can occur all over the body.
- The patches are usually painless but can be itchy.
- They may be lighter or darker than normal skin.
- Usually there are only a few patches but sometimes the skin changes can be extensive, when the condition is known as generalised morphoea.
- Thickened skin may occur in a linear pattern that can affect the head, leg or arm. Unlike morphoea, linear scleroderma can affect not only the skin and fatty tissue but also the underlying muscles and bone, leading to growth deformities in children.
- When linear scleroderma affects the face or scalp, it is known as en coup de sabre, as the scar resembles that caused by a knife or sword wound.
- There is no cure, but in some cases the disease will regress spontaneously.
- The aim of treatment is to stop the inflammation.
- Initial therapy is usually with oral or intravenous steroids. Further treatment to suppress the immune system may be required.
- Physiotherapy is critical for children with linear scleroderma.