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Dr. Mackay is an Assistant Professor of Medicine at Albert Einstein College of Medicine.
"...[O]ne of the most terrible of all human ills [is] to wither slowly...and be 'beaten down and marred and wasted' until one is literally a mummy, encased in an ever-shrinking, slowly contracting skin of steel, [it] is a fate not pictured in any tragedy, ancient or modern"(1)
Hard and tight skin, most often on the face and hands, is the most prominent feature of scleroderma. Also known as Systematic Sclerosis (SSc) and sometimes characterized as the "disease that turns people into stone," scleroderma is especially frightening because we do not know what causes it.
The word "scleroderma" comes from two ancient Greek words: "sclero," meaning hard, and "derma," meaning skin. Yet despite its name, scleroderma can also damage internal tissues and organs and it is this damage which is far more dangerous to a person's health than changes in the skin.
On the positive side, medical researchers have made progress. Better treatments are now available for some of the symptoms of SSc. And, the disease does not appear to be contagious, infectious, cancerous or malignant in any way.
Thankfully, SSc is also rare — affecting approximately 300,000 persons in the United States — although not as rare as better known diseases such as ALS, or Lou Gehrig's Disease, which affects about 30,000 Americans in an average year.
What Exactly is Scleroderma?SSc is a so-called autoimmune — your body reacting against itself — disorder that affects any of three systems: the immune system, the vascular system and the body's connective tissue. Its classic sign is fibrosis, where too much fiber tissue is formed. This is what causes SSc's hardening effect on the skin. Some types of scleroderma are more severe than others. A diagnosis certainly does not mean a death sentence and the disease does not progress relentlessly in all cases. Scleroderma is more common in women than in men and is found in all age groups, though it usually first occurs between the ages of 25 and 55.
How is SSc Detected?Finding out that a person has scleroderma can be a long and frustrating journey. Part of the reason for this is that few doctors see many cases of SSc. Another reason is that the medical definition of SSc is so complex, involving many symptoms that may appear at different times in different forms in different people. And in the early stages, SSc is easily mistaken for other connective tissue diseases, such as systemic lupus erythematosus, polymyositis and rheumatoid arthritis.
Uncertainty and frustration take a toll on the psychological well-being of SSc sufferers, who often describe the period before they are diagnosed as the most difficult part of the illness. To give an idea of the vagueness and complexity of SSc, in 1980 the American College of Rheumatology defined it as the presence of a specific type of skin hardening near the metacarpal phalangeal (MCP) joints of the hand, or two of the following:(2)
The main feature that distinguishes dcSSc from CREST is the extent to which it affects the skin.(3) While both cause skin thickening on the hands and face, dcSSc causes skin thickness on the upper arms, thighs and torso as well. (Figures 1, 2).
Diffuse Cutaneous Scleroderma.
Note the taught, shiny skin over the face and chest, and how the lips have lighter pigmentation (hypopigmentation) and are pursed.
Diffuse Cutaneous Scleroderma.
Note the extensive skin changes on the back.
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