This is an auto-immune disease which affects the blood vessels and the collagen production in the skin. The cause is unknown but it falls within a group of illnesses known as connective tissue disorders.
The term scleroderma simply means hard skin. Many sufferers may have exhibited Raynaud's phenomenon before they develop any signs of scleroderma. The skin signs include a stiffening of the skin of the fingers which becomes shiny and taut. The fingers feel stiff and gradually become less mobile and take on a characteristic appearance called sclerodactyly. The skin can also tighten in other areas such as on the face causing a small mouth. The skin becomes fragile causing dryness and ulcers.
There are two types of scleroderma, - localised and systemic.
In the localised form, there are two distinctions – morphoea in which patches of thickened skin can appear on any area of the body. In linear scleroderma you see lines of thickened skin affecting the head, arm and leg. It can affect the underling muscle and bone causing deformities in children.
In systemic scleroderma there can be widespread involvement of organs. It is divided into localised and diffuse but these terms apply to the distribution of the skin that is affected. Involvement of the internal organs can occur in both types. The bowel is most commonly affected followed by lung, the heart and the kidney. The kidney is rarely involved in the limited type.
Most people with this condition are not severely affected and lead perfectly normal lives. A small proportion die from the illness.
This article published on
26 January 2006
Next review date 01/01/2013
Skin and hair
Skin and hair