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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. A rare disease has such a low prevalence in a population that a doctor in a busy general practice would not expect to see more than one case a year. ...
Medicine In medicine, a persistent and lasting condition is said to be chronic (from Greek chronos). ...
Collagen is the main protein of connective tissue. ...
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. This article is about skin in the biological sense. ...
The blood vessels are part of the circulatory system and function to transport blood throughout the body. ...
In medicine, Raynauds phenomenon is discoloration of the fingers or toes due to emotion or cold in a characteristic pattern in time: white, blue and red. ...
For the network protocol, see finger protocol. ...
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 heart and lungs (from an older edition of Grays Anatomy) The lung is an organ belonging to the respiratory system and interfacing to the circulatory system of air-breathing vertebrates. ...
For the Physics term GUT, please refer to Grand unification theory The gastrointestinal or digestive tract, also referred to as the GI tract or the alimentary canal or the gut, is the system of organs within multicellular animals which takes in food, digests it to extract energy and nutrients, and...
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. Calcinosis is the formation of calcium deposits in any soft tissue. ...
In medicine, Raynauds phenomenon is discoloration of the fingers or toes due to emotion or cold in a characteristic pattern in time: white, blue and red. ...
The esophagus, oe/œsophagus*, or gullet is the muscular tube in vertebrates through which ingested food passes from the mouth area to the stomach. ...
Sclerodactyly is a localized thickening and tightness of the skin of the fingers or toes. ...
Telangiectasias (tee-LAN-jek-TAY-ja) are small enlarged blood vessels near the surface of the skin, usually they measure only a few millimetres. ...
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.
Pathophysiology The cause of the disease is unknown. The overproduction of collagen is thought to result from an autoimmune dysfunction. Autoimmune diseases arise from an overactive immune response of the body against substances and tissues normally present in the body. ...
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. Note: For information about Jason Allen Alexander, the one-time husband of Britney Spears, see Jason Allen Alexander, as well as the relevant subheading in the Britney Spears article. ...
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)
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