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Scleroderma

Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. Progressive systemic scleroderma or systemic sclerosis, the generalised type of the disease, can be fatal. The localised type of the disease tends not to be fatal. more...

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The term 'localised, generalised sclerderma' can be used to describe cases where the disease covers a large area of the body - typically more than 40%.

Signs and symptoms

Scleroderma affects the skin, and in more serious cases, it can affect the blood vessels and internal organs. The most evident symptom is the hardening of the skin and associated scarring. Typically the skin appears reddish or scaly in appearance. Blood vessels may also be more visible. Where large areas are affected, fat and muscle wastage will weaken limbs and affect appearance.

The seriousness of the disease varies hugely between cases. The two most important factors to consider are, the level of internal involvement (beneath the skin), and the total area covered by the disease. For example there are cases where the patient has no more than one or two lesions (affected areas), perhaps covering a few inches. These are less serious cases and tend not to involve the internal bodily functions.

Cases with larger coverage are far more likely to affect the internal tissues and organs. Where an entire limb is affected, symptoms will almost certainly have serious consequences on the use of that limb. The heart and lungs will be affected when the disease covers this area of the torso. Some patients also experience gastrointestinal problems, including heartburn and acid reflux. Internal scarring may sometimes spread beyond what can be seen by the naked eye.

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. (Some physicians consider CREST and limited scleroderma one and the same, others treat them as two separate forms of scleroderma.)

Diffuse scleroderma

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.

Limited scleroderma/CREST syndrome

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:

Read more at Wikipedia.org


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Novel approach: cancer drug might ease scleroderma
From Science News, 11/19/05 by N. Seppa

The chemotherapy drug paclitaxel, when given to mice, shows signs of impeding the skin disease scleroderma, researchers report. By slowing skin thickening, paclitaxel might offer a treatment for a disease that has defied cure.

Scleroderma results when excess collagen protein accumulates in the skin, rendering it fibrous and inflexible. This toughening can cause pain and disfigurement. In some patients, collagen collects in the blood vessels, heart, or lungs, any of which can be lethally damaged. Scleroderma seems to stem from an immune backlash against the body's own tissues, although what triggers the disease is unclear.

Cardiologist Pascal J. Goldschmidt-Clermont of Duke University School of Medicine in Durham, N.C., and his colleagues became interested in paclitaxel, marketed as Taxol, because previous experiments had indicated that the drug could stabilize microtubules--tiny conveyor belts in cells that normally help them function. The researchers had suspected that destabilized microtubules trigger a process that activates the protein TGF beta, a growth factor that stimulates cells to produce excess collagen.

To test whether calming microtubules with paclitaxel would fight collagen buildup, Goldschmidt-Clermont and his team transplanted skin grafts from people with scleroderma into mice. Some transplants were first soaked in paclitaxel, while others weren't. The treated grafts produced significantly less collagen than did the untreated grafts, the researchers report in the December PLoS Medicine.

Paclitaxel also inhibited the activity of TGF beta, the new data suggest.

"This is an intriguing study," says John Varga, a rheumatologist at the Northwestern University School of Medicine in Chicago. In scleroderma research, "targeting TGF beta is where the action is." But Varga cautions that the study is preliminary. "To extrapolate from this mouse model to human scleroderma is hard," he says.

The study authors acknowledge that paclitaxel has one troubling effect. In some cancer patients, it actually causes collagen buildup in the skin. While that risk needs to be further investigated, Goldschmidt-Clermont says, the amount of paclitaxel used in these skin grafts was proportionately much less than that used in patients receiving it as chemotherapy.

Although some drugs can ease lung complications in scleroderma patients, "there isn't any one drug available that treats the disease overall," says Carolyn Weller, vice president of education and research at the Seleroderma Foundation in Danvers, Mass.

If further research indicates a benefit from paclitaxel in people, it would join two other cancer drugs--imatinib mesylate (Gleevec) and rituximab (Rittuxan)--being investigated for use against scleroderma.

Roughly 300,000 people in the United States have scleroderma.

COPYRIGHT 2005 Science Service, Inc.
COPYRIGHT 2005 Gale Group

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