Scleroderma (progressive systemic sclerosis)

Clinical and Differential Diagnosis

Assignment 2

Scleroderma (Progressive Systemic Sclerosis)

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 affec...