Scleroderma
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Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the serious type of the disease, can be fatal. The local type of the disease is not serious.
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Signs and symptoms
Scleroderma affects the skin, and in serious, life-threatening cases, it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin. The seriousness of the disease depends on which organs, if any, are affected. There is discoloration of the hands and feet in response to cold. Most patients (>80%) have Raynaud's phenomenon, a vascular symptom that can affect the fingers, and toes.
Systemic scleroderma and Raynaud's can cause painful ulcers on the fingers or toes, which are known as digital ulcers.
Types
There are three major forms of scleroderma: diffuse, limited (CREST syndrome) and morphea/linear. Diffuse and limited scleroderma are both a systemic disease, whereas the linear/morphea form is localized to the skin.
Diffuse scleroderma is the most severe form - it has a rapid onset, involves more widespread skin hardening, will generally cause much internal organ damage (specifically the lungs and gastrointestinal tract), and is generally more life threatening. The limited form is much milder: it has a slow onset and progression, skin hardening is usually confined to the hands and face, internal organ involvement is less severe, and a much better prognosis is expected.
The limited form is often referred to as "CREST" syndrome. CREST is an acronym for:
These five are the major symptoms of the CREST syndrome. Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of sclerodemra. Morphea/linear scleroderma involve isolated patches of hardened skin - there generally is no internal organ involvement.
Therapy
There is no cure for scleroderma, though there is treatment for some of the symptoms, including drugs that soften the skin and reduce inflammation. Patients often benefit from exposure to heat.
A range of NSAIDs (nonsteroidal anti-inflammatory drugs) can we used to ease symptoms. Naproxen for example. If there is oesophageal dysmotility (in CREST or systemic sclerosis), care must be taken with NSAIDs are they are gastric irritants.
Immunosuppressent drugs, such as mycophenolate mofetil (Cellcept®) are sometimes used to slow the progress.
Digital ulcerations can be helped by prostacyclin (iloprost) infusion. Iloprost being a drug which increases blood flow by relaxing the arterial wall.
Pathophysiology
The cause of the disease is unknown. The overproduction of collagen is thought to result from an autoimmune dysfunction.
Epidemiology
Scleroderma affects approximately 300,000 people in the United States. It is four times as common in women than in men. Incidence rates are estimated at 2-20 per million per year in the United States.
Patients' advocacy
The Scleroderma Foundation is a leading organization dedicated to raising awareness of the disease and assisting those who are afflicted. Its national spokesperson is Jason Alexander.
External links
- Scleroderma Foundation (http://www.scleroderma.org/)
- International Scleroderma Network (http://www.sclero.org/index.html)
- Dermatology Lecture Notes (http://dermind.tripod.com/scleroderma.htm)