Systemic Sclerosis (SSc)
Systemic sclerosis, or scleroderma, is a progressive multi-system auto-immune disease characterized by thickening of the skin, vascular involvement and fibrosis of the internal organs. The cause is really unknown, but the auto-immune processes result in abnormal collagen build-up. They do not really know what triggers that.
The prevalence is about 240 persons per one million. Both the prevalence and the incidence are 4 times greater in women than in men. The onset is between 30 to 40 years of age, and the average 10 year survival rate is now 70-80%.
Here are some links to a couple of different websites- The Scleroderma Foundation and NIH. You can also google the Scleroderma Research Foundation. They have a lot of really great information, booklets, and pamphlets for your patients who have scleroderma.
Subtypes of SSc
There are two subsets of scleroderma- limited cutaneous scleroderma and diffuse cutaneous scleroderma.
Limited Cutaneous Scleroderma
In limited systemic sclerosis, there is gradual skin thickening limited to the distal extremities and the face. There is later involvement of the internal organs. The limited sub-type was formerly referred to as CREST Syndrome which was an acronym that described the symptoms typically seen in scleroderma.
- The 'C' stands for calcinosis. Sub-cutaneous calcium deposits may accumulate most often on the fingers, but also on the forearms, and sometimes on the ischial tuberosities or the lateral malleoli. Pain and infection may result as the deposits become inflamed. They can ulcerate and protrude through the skin or extrude white-ish calcific material.
- 'R' is for Raynaud's phenomenon which is a constriction or narrowing of the blood vessels in response to cold and stress. Severe, untreated Raynaud's may lead to sores and ulcers which can become infected, can heal very slowly or not at all, and can lead to amputations or auto-amputations. Raynaud's phenomenon often one of the first symptoms that people with scleroderma notice.
- 'E' is for esophageal dysfunction and that can include heartburn, dysphagia, reflux. The motility of the esophagus becomes affected.
- 'S' is for sclerodactyly. This is the thickening of the skin on the fingers.
- 'T' is for telangiectasias. Telangiectasias are dilated blood vessels that are near the surface of the skin. They appear as small red or purple spider-like spots, usually on the fingers, palms, lips, face and tongue. They be a cosmetic problem for some people.
