In 1998 my older brother Ron was diagnosed with Scleroderma. At the time, I had never heard of this disease, and most people I talk to today have not heard of it either. Those who have know very little about it, which is really not surprising since it is such a rare disease.
Here are some facts:
Scleroderma, or systemic sclerosis, is a chronic, often progressive, connective tissue disease generally classified as one of the autoimmune rheumatic diseases.
The word “scleroderma” comes from two Greek words: “sclero” meaning hard, and “derma” meaning skin. Hardening of the skin is one of the most visible manifestations of the disease. The disease has been called “progressive systemic sclerosis,” .
Scleroderma is a disease whose symptoms may be visible, as is the case when the skin is affected, or the symptoms may be invisible, as when internal organs are affected.
Scleroderma is not contagious, infectious, cancerous or malignant.
The symptoms of Scleroderma vary greatly from individual to individual, and the effects of Scleroderma can range from very mild to life-threatening. The seriousness will depend on what parts of the body are affected and the extent to which they are affected.
It is estimated that there are approximately 300,000 persons with Scleroderma in the United States, including 80,000 to 100,000 with the systemic form and the rest with the localized form. Statistically, approximately three to four times more women than men develop the disease. Scleroderma can develop and is found in every age group from infants to the elderly, but its onset is most frequent between the ages of 25 to 55.
Factors other than sex, such as race and ethnic background, may influence the risk of getting Scleroderma, the age of onset, and the pattern or severity of internal organ involvement. The reasons for this are not clear.
The exact cause or causes of Scleroderma are still unknown.
There is no cure.
On June 14, 2004, my brother lost his battle with this disease.
Eighty percent of scleroderma patients are female, typically diagnosed in their childbearing years. The average time from symptom onset to correct diagnosis of scleroderma is three years. Many patients ignore an early symptom of the disease, Raynaud’s phenomenon, a sensitivity to cold in the hands and feet which affects 5 to 10% of the U.S. population. It is estimated that up to 75% of the people with scleroderma have yet to be diagnosed, and although there is no known cure, early treatment is essential.
Scleroderma can be localized or systemic. Localized Scleroderma appears in the hands or joints, making skin hard and shiny, and causing mobility to be more difficult. Systemic Scleroderma affects not only skin and joints, but hardens internal organs, which can be fatal.
The most common symptoms of Systemic Scleroderma are often referred to as CREST, an acronym that stands for Calcinosis, Raynaud’s phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasias.
Calcinosis (KAL-sin-OH-sis): areas of calcium deposits in the connective tissues, typically found on the fingers, hands, face, and body. When the deposits break through the skin, painful ulcers can result.
Raynaud's (ray-NOHZ) phenomenon: very cold hands and toes. About 70% of scleroderma patients recognize this as a symptom before anything else. As the vessels contract, the hands or feet turn white and cold, then blue. As blood flow returns, they become red. Fingertip tissues may suffer damage, leading to ulcers, scars, or gangrene.
Most scleroderma patients wear cotton gloves indoors as they just cannot seem to get their fingers warm. Air conditioning in restaurants and theaters is difficult for them.
Esophageal (eh-SOFF-uh-GEE-ul) dysfunction: impaired function of the esophagus, the result of which can be swallowing difficulties and chronic heartburn or inflammation.
Sclerodactyly (SKLER-oh-DAK-till-ee): thick and tight skin on the fingers, resulting from deposits of excess collagen within skin layers. The condition makes it harder to bend or straighten the fingers. The skin may also appear shiny and darkened, and be accompanied by hair loss. The skin on the fingers, hands and arms is tight, hard, and cannot be pinched or moved The skin will appear very shiny, especially on the hands and arms.
Telangiectasias (tel-AN-jee-ek-TAY-zee-uhs): small red spots on the hands and face that are caused by the swelling of tiny blood vessels. While not painful, these red spots can create cosmetic problems.
June is National Scleroderma Awareness month.
Visit the Scleroderma Foundation website at
Scleroderma.org
and learn more about scleroderma and the many ways you participate in Scleroderma Awareness month.
If you have some of the symptoms often associated with scleroderma or recognized them in someone you know, please view the Scleroderma website at
Scleroderma.org
or call the National Scleroderma office for information at 800-722-HOPE.
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