Diffuse cutaneous systemic sclerosis
This is systemic disorder characterised by progressive accumulation of collagen and fibrosis. The skin is most commonly affected, appearing hard, swollen and closely tethered. Other locales such as the vascular, respiratory and gastro-intestinal systems are frequently involved. Death from renal failure, cardiac failure, pulmonary insufficiency or intestinal malabsorption may occur.
Females are affected more than men in the ratio 4:1. Presentation is usually under 50 years of age.
The features of the different forms of systemic sclerosis have been described (1):
Reference:
- 1) Arthritis Research Campaign (2010). Topical Reviews - Systemic sclerosis and related disorders.
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