What is scleroderma?

Scleroderma is an unusual form of arthritis described as "hardening of the skin" as this is a part of the body affected. There are two major forms - systemic and localised.

Systemic scleroderma

Systemic forms of scleroderma come in two forms as well - diffused systemic sclerosis (SS) and limited systemic sclerosis. SS indicates the form of scleroderma with more generalised skin involvement as well as body systems such as the oesophagus, joints, intestines, lungs, heart and kidneys.

It varies greatly in severity and or progression of the disease. It can range from the widespread thickening of skin (diffuse) to a more limited skin involvement (CREST).

Diffuse scleroderma is another term which describes systemic sclerosis and skin changes in many parts of the body. Tight, glossy skin can be present on the trunk and upper arms, as well as the face, chest and extremities.

CREST scleroderma

CREST is an acronym made up of the first letters of the five most prominent manifestations of this form of scleroderma:

• Calcinosis (the accumulation of calcium salts under the skin)
• Raynaud's phenonemon (which affects the circulation of the small blood vessels primarily in the feet and hands)
• oEsophageal dysfunction (the loss of normal action in the lower oesophagus)
• Sclerodactyly (the skin on the digits becomes hard)
• Telangiectasia (the appearance of small blood vessels near the surface of the skin).

Localised forms of scleroderma

This includes morphea, a localised scleroderma, which begins with an inflammatory stage followed by the appearance of one or many patches or plaques, and linear scleroderma, which is a band of thickening of the skin often limited to one area.

The outlook for plaque-type morphea is usually for it to become less active after a few years, with skin softening and staining, and possibly some sunken patches of skin. The outlook for linear morphea is for it to take longer to improve, and there may be calcium deposits in the skin lesions. In severe cases there may be muscle wasting in the limbs and these may then become stiff and weak.

What causes scleroderma?

No one knows what causes scleroderma but it is found in all countries and ethnic groups, with patients ranging from infants to the elderly. It is four times more common in women than men and its frequency in both sexes increases with age.

While seemingly rare, scleroderma is in fact far more prevalent than muscular dystrophy, multiple sclerosis and a host of other better known and understood diseases.

What treatment is there for scleroderma?

While there is no specific treatment for scleroderma, there are many treatments which can control the fibrosis of the skin and internal organs and help control the symptoms. As with other forms of arthritis, there are many things a person can do to assist the management of their disease.

Patients should take special care to protect their hands, face and ears from the cold or overly active air-conditioning. Other "common sense" measures include eating a balanced diet, avoiding alcohol and not smoking, maintaining a sensible weight, and avoiding fatigue.

Further information

Arthritis New Zealand provides specialist newsletters for people with scleroderma (contact details further below).

Original material provided by Arthritis New Zealand. Edited by everybody, July 2005.