Scleredoma variants

Scleredoma – Not Just the Excessive Deposit of Collagen.

Scleroderma is a chronic auto-immune disease characterized by excessive deposits of collagen, causing inflammation and thickening of the skin and is a relatively rare disease. Typically, this is induced by the body’s tissues being attacked by its own immune system, formating scar tissue (fibrosis) in the skin and organs of the body. Overlaps of scleroderma and other connective tissue diseases, such as rheumatoid arthritis can occur. Scleroderma is expected to affect at least 300,000 people in the United States, is four times more common in women than in men, and it’s cause is unknown, although there is evidence that genetics at least plays a partial role.

Scleroderma in most cases, affects the skin and is known for hardening of these areas with associated scarring. This will give a reddish or scaly appearance. Blood vessels may also become more visible, wasting of fat and muscle with weakened limbs, also in large affected areas the appearance of the limbs is altered.

There are three major types of scleroderma. They are diffuse, limited and morphea sometimes called linear.

Diffuse scleroderma, also known as progressive systemic sclerosis, sometimes fatal, involves symmetric thickening of skin of the extremities, face, and trunk (chest, back, abdomen, or flanks) which can rapidly progress to hardening after an early inflammatory phase. Diffuse scleroderma will cause severe internal organ damage to the lungs and gastrointestinal tract, obviously the more life threatening variant. Organ disease can occur early on and those affected can include: the esophagus, bowels, lungs with scarring (fibrosis), heart, and kidneys. High blood pressure can be a troublesome side effect.

The second kind, limited scleroderma is known as CREST syndrome. CREST is an acronym that stands for:

Calcinosis – the formation of tiny of calcium in the joints and is common with systemic scleroderma sufferers, often localized to the elbows, knees and other joints.

Raynaud’s Phenomenon – In most serious cases, scleroderma can affect blood vessels, including the tiny arterioles of the finger tips, toes, causing tiny ulcers or blackened (dead) skin. These vessels can have a tendency to spasm when the areas are exposed to cold, leading to blueness, whiteness, and redness of involved fingers, toes, and sometimes nose or ears. This vascular condition is referred to as Raynaud’s phenomenon and is also a symptom of arthritis. The extremities can also experience an associated “tingling” sensation.

Esophageal dysmotility – characterized by poorly functioning muscle of the lower two-thirds of the esophagus.

Sclerodactyly – the localized thickening and tightness of the skin of the fingers or toes.

Telangiectasia – tiny red areas, most often on the face, hands and in the mouth behind the lips. These areas blanch when they are pressed upon and look like dilated capillaries.

CREST is a systemic disease and is much milder,having a slow process. The skin hardening is usually just on the face and hands and internal organ progression is less severe. The prognosis is much more positive for limited scleroderma.

The third and final variant, morphea sometimes called linear, is scleroderma skin that is localized to a patchy area of the skin that becomes hardened and slightly pigmented. Sometimes morphea can cause multiple lesions in the skin. Morphea is not associated with disease elsewhere in the body. Linear scleroderma is scleroderma that is localized usually to a lower extremity, frequently presenting as a strip of hardening skin down the leg of a child. Linear scleroderma in children can stunt bone growth of the affected limb. Sometimes linear scleroderma is associated with a “satellite” area of a patch of localized scleroderma skin, such as on the abdomen.

Although there is no cure for this disease, there is treatment for some of the symptoms. These treatments often involve drugs that soften the skin and reduce the swelling. Having heat exposure will also help most of sufferers. You need to speak to your doctor about more specific information on this disease.

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This entry was posted on Thursday, August 4th, 2011 at 4:56 pm and is filed under Vintage Electronics. You can follow any responses to this entry through the RSS 2.0 feed. Both comments and pings are currently closed.