The forms of scleroderma
There are two major types of Systemic Sclerosis:
1. Limited Scleroderma also known as CREST syndrome:
C= Calcinosis cutis (calcium deposits under the skin)
R= Raynaud (finger discoloration upon exposure to cold)
E= Esophageal dysmotlity (disorder affecting 2/3 of the lower esophagus)
S= Sclerodactyly (thick, tight skin of the fingers; – pudgy fingers)
T= Telangiectasia (tiny dilated blood vessels visible on the face and hands)
2. Diffuse Scleroderma
The distinction between these two types of scleroderma is based on the pattern and severity of skin and internal organ involvement, which is more extensive and has a more rapid progression in the diffuse type. The skin, digestive system, heart, lungs and kidneys are the most commonly affected organs which may be negatively impacted by the potentially serious complications of scleroderma. At first, these complications may go unnoticed.
Systemic sclerosis does not only affect the body and internal organs, but also greatly reduces the quality of life of patients, who often experience pain, fatigue and shortness of breath in their everyday activities. It is still unknown what exactly triggers this autoimmune disease.
Who can develop Scleroderma?
- Scleroderma is found throughout the world.
- It is estimated that several thousands of people live with SSc in Quebec.
- In Quebec, SSc is five times more common in women than in men.
- Onset usually occurs in the fourth decade of life although the disease can also strike children and people of all age groups.
- Scleroderma is not contagious.
- SSc of both types is associated with a significant reduction in life expectancy.