Deadly Skin Disease

By: Sharon Bell

It's neither contagious nor cancerous. But this disturbing skin condition can greatly affect your self-esteem and make it difficult for you to perform your daily activities. In case you're wondering what I'm talking about, it's scleroderma - a rare disease that can run in families.

Scleroderma is a disease in which the skin and other body parts degenerate, thicken and become stiff. This disorder not only affects the skin but the joints, diges?tive system, heart, kidney, lungs, fingers and toes as well. Anyone can get the disease but it is common in women between 30 and 50 years old.

The exact cause of this disease is unknown but scleroderma is believed to be an autoimmune condition wherein the body's immune system attacks its own tissues. The risk factors are likewise unknown and the disease can't be prevented at present.

"Scleroderma (progressive systemic sclerosis) is a disorder characterized by excessive buildup of fibrous connective tissue. Initially scleroderma, which means thickening (sclero) of the skin (derma), was thought to involve only the skin. Recently, however, it has been recognized that vital internal organs also may be tar?gets of increased collagen deposits. Hence, the term progressive systemic sclerosis (PSS) is more accurate," according to Dr. Israeli Jaffe, professor of clinical medicine, in "The Columbia University College of Physi?cians and Surgeons Complete Home Medical Guide."

What happens here is that there is a continuous produc?tion of fibrous connective tissue or collagen. This gelati?nous protein is made by cells called fibroblasts which, for some strange reason, go out of control.

"For example, when we cut ourselves, the wound heals with new connective tissue and new overlying skin. Once the cut is healed, the reparative process stops and new collagen ceases to be produced. In con?trast, in scleroderma or PSS, fibrous tissue is produced at an accelerated rate, even without the stimulus of a wound. As a result, increasing amounts of young or immature collagen are continuously being laid down in the involved tissues, replacing the normal cells with connective tissue, as though it were a scar. This makes the skin, for example, thick and tight as the normal clastic tissue is replaced by dense fibrous tissue. Hair growth and sweating generally stop as the hair fol?licles and sweat glands are destroyed. Often the patient may experience intense itching as normal skin struc?tures are replaced by excessive collagen," Jaffee ex?plained.

Among the complications of scleroderma are poor wound healing and gangrene, bleeding tendencies, congestive heart failure, kidney failure, high blood pressure and lung destruction.

"Scleroderma leaves the esophagus stiff and inflex?ible so that it doesn't propel the food down to the stomach as it normally does. When the organs adjacent to the food pipe become enlarged, they may press on and narrow it," said Dr. Isadore Rosenfeld of the New York Hospital - Cornell Medical Center in "Symptoms."

"When PSS attacks internal organs, the disease may be life-threatening. In the lung, for example, PSS may cause an increase in connective tissue in the delicate air sacs, a condition called pulmonary fibrosis. The normal process of oxygen transfer from the inhaled air to the blood is progressively blocked. As a result, blood is deprived of its normal oxygen content, leading to shortness of breath, which is often aggravated by re?striction in chest wall movement because of thickening of skin over the chest," Jaffe added.

Unfortunately, there is no cure for the disease and patients may succumb to complications in five years. With the right treatment, however, symptoms can be controlled.

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