About Scleroderma

Scleroderma is an autoimmune connective tissue disease affecting blood vessels and collagen production. It is more common in women than men. The cause is still largely unknown and although there is no cure, there are many treatments to slow down or halt disease progression. The word scleroderma, which means "hard skin", is the generic term describing a group of related diseases in which the skin becomes hardened and sclerotic. There are two kinds of scleroderma: systemic sclerosis which affects the internal organs as well as the skin, and localised scleroderma which affects a specific area of skin but not the organs.

LOCALISED SCLERODERMA
Localised scleroderma (not to be confused with limited) indicates that this form of scleroderma is confined to the skin only and does not affect the internal organs. It is more common in children. There are two main types, morphoea and linear.
Morphoea
Patches of thickened skin, which may be rounded or oval in shape, can occur all over the body. They are usually painless but can be itchy. The patches 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.
Linear scleroderma
Thickened skin may occur in a linear pattern which 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.

SYSTEMIC SCLEROSIS
The term systemic means that the scleroderma affects the internal organs. The terms limited and diffuse refer to the extent of skin involvement.

Limited systemic sclerosis (lcSSc) is also sometimes referred to as CREST syndrome, which stands for calcinosis, Raynaud's Phenomenon, (o)esophageal dysfunction, sclerodactyly and telangiectasia. Skin involvement is limited to the face, forearms, hands and lower legs, whereas in diffuse disease the skin changes can affect the whole body. The severity and problems created by skin changes will vary greatly between patients. Problems include tightening of the skin around the fingers, the face and other areas of the body causing contractures (fixed joints) and a small mouth (microstomia), ulceration, dryness and irritation, broken blood vessels (telangiectasia) on the face and hands and calcinosis protruding through the skin. In addition to drug therapy, physiotherapy and exercises are important. Warm paraffin wax baths can also be useful and moisturising the skin is essential.

The internal organs can be affected in both limited and diffuse disease. Gastrointestinal (gut) involvement affects most patients and investigations will determine which treatment will be most effective.
Heart and lung involvement can also be associated with both forms, although the heart is not as commonly affected as the lung. Lung fibrosis is more common in diffuse patients and provided it is diagnosed early enough, progression can be halted or slowed down.
A small percentage (15%) of patients with lcSSc will develop pulmonary hypertension (PHT) a condition affecting the vessels taking blood from the right side of the heart to the lungs. These patients need to be seen by a cardiologist.
The kidneys are rarely affected in limited disease, however approximately 5% - 10% of diffuse patients will incur some form of renal involvement. It is very important for all patients to have their blood pressure checked.

For a very small minority the complications can be so severe that the disease is fatal. However the overwhelming majority of people with Systemic Sclerosis can lead full and productive lives.

All patients with Scleroderma should be closely followed up and have annual tests for organ problems.

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