What is Scleroderma?
The term Scleroderma literally means “hard skin”. It is an autoimmune disease in the same group of diseases as SLE (Systemic lupus Erythematosus). It is a 'chronic, generalised connective tissue disorder. It actually encompasses a spectrum of disorders in which there are changes in the skin, joints and internal organs, due to the abnormal changes of connective tissue [which is the supporting structure of skin and internal organs]. As well as an increase in deposition of collagen, which thickens the skin, there are abnormalities in the circulation (capillaries and arteries) in the periphery of the body.
The term “systemic sclerosis” is more accurate when other parts of the body are affected. The diagnosis of systemic sclerosis should be doubted in the absence of Raynaud’s phenomenon.
Prevalence and incidence
It affects women more than men (female to male ratio = 4:1), it is not an inherited condition. The peak age is in the 4th and 5th decades, though it can occur in children. 10 to 20 people per 100,000 suffer from it, so it is very rare.
What is the cause?
Scleroderma has no known cause. It is thought that the immune system stimulates the collagen-producing cells (fibroblasts) to produce too much collagen.
There may be an association with certain agents, and the person may be predisposed to developing the disease after a trigger. For example, an infection may set it off. It may be associated with silica dust, quartz, adhesive or coating agents, PVC and trichlorethylene. There may be some link between scleroderma and the presence of female hormones.
Amended November 2008
