Systemic sclerosis

An autoimmune disease of unknown cause, systemic sclerosis is 3–4 times more common in women than in men and usually starts between the ages of 30–40 (although later in men) (New Zealand Dermatological Society Incorporated, 2009). It is a multisystem disease in which vasculopathy of small arteries produce symptoms affecting the gastrointestinal (GI) tract, lungs, kidneys, heart, liver, nervous and musculoskeletal systems (Graham-Brown and Burns, 2006). The typical skin symptoms are created by ‘thickening of the skin’; there is also destruction of the hair follicles and sweat glands. Box 13.2 lists these symptoms. Note that a milder form of the condition is called CREST. When there is no systemic disease but skin sclerosis is present this is known as morphea.