Collagen Build-up: Scleroderma
Scleroderma is a rare, chronic disease characterized by excessive deposits of collagen. It affects approximately 300,000 people in the United States. Scleroderma is four times as common in women as it is in men. Progressive systemic scleroderma, also known as systemic sclerosis, is the generalized type of the disease and can be fatal.
Scleroderma tends to affect the skin. The most evident symptom is the hardening of the skin and associated scarring, giving off a reddish or scaly appearance. Blood vessels may also tend to be more visible. Fat and muscle wastage will weaken the limbs and affect their appearance when larger areas are affected. In more serious cases, scleroderma can affect the blood vessels.
The seriousness of scleroderma varies between cases. The two most important factors to consider are the level of internal involvement that occurs beneath the skin and the total area covered by the disease. A patient may have only one or two affected areas or lesions or they may have many larger ones. Most patients of scleroderma have Raynaud’s phenomenon, which is an arthritic vascular symptom that can affect the fingers and toes. In fact, the hands and feet may have a discoloration in response to cold. Raynaud’s phenomenon and systemic scleroderma can cause painful ulcers on the fingers or toes. Calcinosis, or the deposit of calcium around the joints, is also common for sufferers of systemic scleroderma and can often be found near the elbows, knees or other joints.
There are three major types of scleroderma. They are diffuse, limited and morphea, or linear. The first type, diffuse scleroderma, is a systemic disease. It is the most severe form. It is characterized by a rapid onset and involves more widespread skin hardening. Diffuse scleroderma will generally cause severe internal organ damage, specifically to the lungs and gastrointestinal tract. It is more life threatening.
The second kind, limited scleroderma, is also known as CREST syndrome. CREST is an acronym that stands for for “Calcinosis, Raynaud’s syndrome, Esophageal dysmotility, Sclerodactyly, and Telangiectasia,” which are the five major symptoms of limited scleroderma. CREST is also a systemic disease, but is much milder. It has a slow onset and progression. Skin hardening is usually just in the face and the hands and internal organ involvement is less severe. The prognosis is much better for limited scleroderma.
The last kind of scleroderma is Morphea, or linear scleroderma. This type generally does not have any internal organs affected. It just involves isolated patches of hardened skin. It is the least severe type of scleroderma.
There is no cure for this disease. However, there is treatment for some of its symptoms. These treatments include drugs that soften the skin lesions and reduce inflammation. Heat exposure has been known to help some patients. Ask your doctor for more information.
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