Derived from the Greek words “sclerosis” meaning hardness and “derma” meaning skin, scleroderma literally means hard skin. Scleroderma, also known as systemic sclerosis, is a progressive and chronic connective tissue disorder. Some unknown factor triggers the over-production of collagen (body protein) causing thickening, hardening and scarring of the skin and internal organs. Normally collagen keeps the skin soft, but the overproduction makes the affected tissue thick and hard. This, in turn, affects the amount of blood the small vessels carry to many parts of the body.
The most characteristic feature of scleroderma is the presentation of hard, hide-bound thickening of the skin. Less visible but of major importance are the lesions that occur in small blood vessels (vascular lesions), which may involve major organs.
The natural history, or course of scleroderma, varies widely from that of a mild nuisance to a severe multi-system disease. Forms of the disease that primarily affect the skin without major organ involvement, have better long-term outlook. Those forms of scleroderma that involve major organs, such as the heart and kidney, are potentially more severe with possibly less desirable long-term outcomes.
Scleroderma is an autoimmune disease which means that it is a condition in which the body’s immune system attacks its own tissues. The normal role of the immune system is to provide protection from invaders such as viruses. In autoimmune disorders, the ability to distinguish foreign from self is compromised. As immune cells attack the body’s own tissue, inflammation and damage result. Scleroderma can vary a great deal in terms of severity. For some, it is a mild condition; for others it can be life-threatening. There are medications to slow down disease progression and help with symptoms, but as of yet, there is no cure for scleroderma.
The National Institute of Arthritis and Musculoskeletal and Skin Disease, recognizes that although scleroderma is often referred to as if it were a single disease, in fact, it is really a symptom of a group of diseases that involve the irregular growth of collagen. Collagen forms the cellular matrix of connective tissue found in skin, tendons, ligaments, cartilage, bone, blood vessels, the gut and other internal organ surfaces. In some forms of scleroderma, hard, tight skin is the extent of this irregular collagen over-production process. In other forms, however, the problem goes much deeper, and may severely affect blood vessels and internal organs such as the heart, lungs, and kidneys.
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