Scleroderma is essentially a chronic autoimmune disorder characterised by the deposition of collagen in many tissues throughout the body, typically the skin, causing it to harden and thicken. It is an uncommon disease affecting 8 to 9 people per 100,000 in Sydney, which is comparable with prevalence rates overseas. Scleroderma normally affects middle aged women, with the usual age of onset being 45 to 65 and an incidence ratio of 3 women to every man.
The aetiology is thought to be multifactorial, including environmental factors such as a virus and/or toxin, and genetic predisposition. Although the dysfunction occurring in scleroderma is well understood, the trigger for these abnormalities is poorly understood.
Scleroderma can be divided into localised and systemic types.
· The localised form is limited to the skin, primarily affecting the hands and face.
· The systemic type is more serious, affecting the skin more extensively and also involving the connective tissue of internal structures, especially the lungs, digestive tract and kidneys.
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In both types the hands of the patient are usually affected first, progressing to the arms, face, and feet, the trunk and legs then becoming affected in the systemic type. The skin becomes dry and leathery as it thickens and stiffens, attaching firmly to the subcutis. This causes shrinkage of the skin and the patient then feels as if encased in a shell that is too small for them. The face develops the characteristic impassive expression due to the skin tightening, decreased oral aperture and thin lips are also observed. The deposition of collagen in the skin causes the hair to thin and impairs sweating. About half of patients present initially with joints that resemble early rheumatoid arthritis.
Raynaud’s phenomenon occurs in almost all cases of scleroderma. This manifests clinically as a well demarcated colour change of the extremities – the finger and toe tips become white, then blue and finally red – in accordance with the lack of blood circulating to the extremities. The terminal phalanges often suffer gangrene, ulcers and a burning or numb feeling as a result.
CREST syndrome is an acronym often used to describe the pattern of symptoms seen in cases of scleroderma.
It refers to:
Scleroderma usually progresses slowly over a number of years, sometimes with remissions or pauses in progression but eventually it does tend to become more and more extensive. Rarely it can follow a rapid course with severe systemic involvement within a period of months. Treatment is difficult because patients do not respond well to steroids or immunosuppressants. D-penicllamine has helped some patients especially those with the more serious form. Symptomatic treatment depends on the presenting symptoms. The ten year survival rate is 33%, higher for the systemic type (55% or 70% depending on the extent of visceral involvement). If malignant hypertension complicates scleroderma the prognosis is very poor.
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