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What is Scleroderma? By Amy, Daughter of Systemic Sclerosis Patient

Imagine for me, if you would, that in certain areas of your body the skin has begun to thicken and harden. Then on top of this, you have a painful sensitivity to cold, frequent heartburn, stiff joints and various other internal problems.

These are the things that my father has to deal with daily due to a rare disease known as scleroderma, which, when literally translated, means the hardening of the skin. I am here today to inform you of some of the facts surrounding scleroderma, the types of scleroderma and the support groups affiliated with scleroderma. Scleroderma is a chronic autoimmune disease of the connective tissue.

Generally, it is classified as a rheumatic disease that, in the systemic form, affects an estimated eighty thousand to one hundred thousand people in the United States. If one were to count the number of people with Raynaud's Phenomenon (which I?ll tell you about later) and at least one symptom of scleroderma, the count would reach nearly one hundred and fifty thousand, and there are even more carrying the localized form of the disease.

Overall, female patients outnumber the men, four to one. The average age of diagnosis is in the forties, although there are many exceptions in scleroderma as it is found not only in children, but also in elderly patients. The exact cause of scleroderma is unknown, but doctors have found that scleroderma involves an overproduction of collagen. Depending on the form of scleroderma, the effects may either be visible to the open eye, or invisible and only seen internally.

Scleroderma is a highly individualized disease with symptoms that may range anywhere from mild to potentially life-threatening. There is no cure for this disease, but there are many treatments for those afflicted. While the bad aspects of scleroderma definitely outweigh the good, scleroderma is not contagious, cancerous or malignant, or inherited, generally speaking. There are two different types of scleroderma: localized and systemic.

Systemic sclerosis is the more common and deadly form of scleroderma in which the immune system damages two main body areas; the small blood vessels and the collagen-producing cells located in the skin and throughout the body. These areas include the connective tissue in many parts of the body such as the skin, esophagus, gastrointestinal tract, lungs, kidneys, and heart. They also may include the muscles and joints in the body.

There are many effects of this form of scleroderma. First and foremost, nearly all scleroderma patients are cold sensitive. This is a result of the blood vessels in the fingers narrowing and sometimes, completely closing off the channel of blood in the fingers. This often causes small cuts on the hands to heal very slowly. Because of this decreased blood supply, the majority of scleroderma patients are also very sensitive to cold temperatures. This vascular part of the disease is the cause of Raynaud's phenomenon that afflicts approximately ninety percent of those with systemic sclerosis. Raynaud's phenomenon causes fingers to change color with exposure to cold temperatures.

The overproduction of collagen is the part of scleroderma that causes the thick and tight skin, lung problems, gastrointestinal problems, and the heart problems associated with this form of the disease. When a body is functioning normally, the immune system sends out signals to the collagen producing cells to form a scar after an injury or infection has been cleared. When one has scleroderma, scar tissue is produced, for no apparent reason, and simply builds on the skin and other internal organs.

Systemic sclerosis can also be divided into two categories known as limited and diffuse. These two categories refer to the amount of skin involvement. While both forms produce internal organ damage, the limited form has a tendency to be less severe. The limited form is often referred to as the CREST form. CREST stands for calcinosis, or calcium deposits in the skin; Raynaud's phenomenon, which I discussed earlier; esophageal dysfunction, or acid in the esophagus felt as heartburn; sclerodactyly, the thick and tight skin of the fingers; and telangiectasias, a particular type of red spots that may appear on the skin.

Localized scleroderma, more commonly affects children. This type of scleroderma is usually only found in a few places on the skin or in the muscles. While this form of scleroderma does not affect any internal organs, it is based in the collagen-producing cells in some areas of the skin instead of affecting all areas. This form is confined to a local area of skin and tissue. Localized scleroderma is generally associated with thick patches of skin, morphea, or with linear scleroderma, which is a line of thickened skin that extends down one of the extremities. This type of scleroderma rarely, if ever, develops into systemic sclerosis.

If you, a family member, or a friend has scleroderma, there are many support groups available to help one deal with the emotional effects of this disease. The sclero.org website provides links to other websites affiliated with scleroderma and stories of patients as they tell of their connection with the disease as well as many facts that may help people understand this rare disease.

A few years ago, Bob Saget, from America's Funniest Home Videos, made a television movie called For Hope as a tribute to his sister who died of scleroderma. I saw this movie when it came out, though it was before my father had been diagnosed. I think that if you?d like to get a first-hand look at what scleroderma can do, this would be a worthwhile movie to obtain.

In conclusion, I hope that I have helped you understand a little more about this disease. You should know; however, that not all patients suffer as horribly as I may have made it sound. The affects of scleroderma may vary from patient to patient. If more people were made aware of this disease, maybe more support would be given to the research involved in finding a cure for those afflicted.

Remember this: though it is not a widespread disease that you will read about in the media, it still affects hundreds of thousands daily. Diagnosed in 1995, I have watched my father deal with the affects of this disease daily, never knowing what the outcome is going to be.

To Contact the Author

This Story is Featured in Voices of Scleroderma Volume 1

The story on this page is featured in the top-notch Voices of Scleroderma Volume 1 which features articles by top scleroderma experts, including Dr. Joseph Korn, as well as 100 true patient and caregiver stories from this website! The book delivers excellent information and support for scleroderma patients and caregivers. It's a great way to inform family and friends about scleroderma, too!

SCLERO.ORG is the world leader for trustworthy research, support, education and awareness for scleroderma and related illnesses, such as pulmonary hypertension. We are a service of the nonprofit International Scleroderma Network (ISN), which is a 501(c)(3) U.S.-based public charitable foundation, established in 2002. Meet Our Team, Volunteer, or Donate.

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