When Mary noticed her skin tightening and thickening uncomfortably on her face and hands, she went to her primary care physician to get answers. There were other symptoms she had noticed, as well, and showed him the small, widened blood vessels near the surface of her skin—telangiectasia. Although Mary had visited a medical spa for several laser treatments to remove them, the telangiectasia always returned. Then there was the mysterious itching that never stopped. Mary could not identify the source, because there were no hives, bites or other visible causes. Her physician recognized that these symptoms could be pointing to the onset of an autoimmune disease and referred her to a rheumatologist. Lab tests were ordered and soon, Mary received the diagnosis of scleroderma.
What is scleroderma?
According to the Scleroderma Research Foundation website (www.srfcure.org), scleroderma, or systemic sclerosis, is a chronic connective tissue autoimmune disease. The word “scleroderma” comes from two Greek words: sclero meaning “hard” and derma meaning “skin.” Hardening and tightening of the skin are two of the most visible manifestations of the disease. Scleroderma, however, can be much more than this. Peripheral vascular damage can occur and, in some cases, the joints and muscles are affected, resulting in a loss of mobility. Scleroderma can produce chronic, complex and debilitating symptoms, often damaging internal organs with life-threatening consequences. Systemic scleroderma can damage the lungs, kidneys and gastrointestinal tract with deadly results. It can create deleterious effects throughout the body, and it falls under the heading of a vascular disease because it can constrict and injure tiny blood vessels. It is identified as a connective tissue disease because it can cause abnormal changes to the skin, tendons and bones and, like many other rheumatic disorders, scleroderma is believed to be an autoimmune disease because it can apparently trigger the body into a state of disregulation.
According to the same site, the course of the disease is unpredictable, and its symptoms and severity vary from one person to another. Women are most commonly afflicted with scleroderma; some estimates suggest as many as four out of every five patients are female, and the disease is most frequent between the ages of 20–50.