Massage & Bodywork

November/December 2009

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PATHOLOGY PERSPECTIVES and small blood vessels are activated. These cells begin to produce enormous amounts of collagen and other connective tissue matrix. This material is then deposited in the interstitial spaces and in the intima of the affected blood vessels. This usually happens in the skin, but other tissues may be affected as well. Further, the disruption of the innermost layer of small blood vessels can impede blood flow to vital tissues—for instance, the motor nerves that supply the digestive tract. From this description, it is clear that scleroderma is a serious, even life-threatening disease. The good news is that it doesn't always occur in the worst possible form, and for many people it is quite mild. Some patients find that their disease progresses for several years, then stabilizes for several years, and then mysteriously resolves, never to occur again. TYPES OF SCLERODERMA Scleroderma is classified into two main groups, each with subtypes. localizEd SclErodErMa This affects the skin only and is not associated with organ dysfunction. Morphea Scleroderma Oval-shaped patches appear on the skin of the extremities, although the trunk is occasionally involved. These are often pale in the middle and purplish around the edges. These areas are hard and inelastic, and the skin may appear shiny and thickened. Ulcerations may occur as well. linear Scleroderma This form involves streaks of hardened skin, often on the face or neck. A particular facial lesion is sometimes called coup de sabre or sabre cut for its scar-like appearance on the face. SySTEMic This usually affects the skin, but internal organs are also sites of collagen deposition. limited cutaneous Systemic Scleroderma This condition mostly involves the skin, but the gastrointestinal tract is also frequently involved. The acronym CREST, described below, was coined for the symptomatic profile seen with this version of scleroderma. diffuse cutaneous Systemic Scleroderma This can involve the skin and all the internal organs, but frequently focuses on the lungs, heart, and kidneys. This is a severely threatening form of the disease, investing vital organs with deposits of useless scar tissue that interferes with their function. Sine Scleroderma Sine means without. In this rare form the skin is spared, but the internal organs are invested with connective tissue that interferes with their ability to function normally. CREST SYNDROME/ ABCDCREST SYNDROME CREST syndrome, discussed previously, is an acronym for the symptomatic profile that is most commonly seen with limited cutaneous systemic scleroderma. It stands for calcinosis; Raynaud phenomenon; esophageal dysmobility; sclerodactyly; and telangiectasias. In 2004, a group of specialists convened to gather information about cutaneous systemic scleroderma, and revised the acronym to this: ABCDCREST. They found that if a patient has any three or more of these nine signs, the chance of their having this form of scleroderma is about 99 percent, making this a far more accurate test than many of the intrusive procedures people typically go through on their way to a definitive diagnosis. a—auToanTibodiES This refers to the presence of distinguishable antibodies in blood tests that indicate a tendency toward autoimmune disease. b—bibaSilar PulMonary FibroSiS This indicates the deposition of collagen fibers in the lungs, which reduces lung function and increases the risk of infection. This may be due to Constriction of vessel decreases blood supply to fingers, causing them to turn pale Cut-away view of vessel Raynaud's phenomenon: vasoconstriction in the extremities. Medical Illustration Copyright © 2009 Nucleus Medical Art. All Rights Reserved. www.nucleusinc.om. 108 massage & bodywork november/december 2009

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