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Scleroderma is a condition in which the skin gradually tightens, thickens, and becomes hard; thus losing its ability to stretch. The condition is considered a connective-tissue disorder as well as a rheumatic condition. The disease most often affects the hands, face, and feet. However, blood vessels can also be affected which results in restricted blood flow that can damage the body’s digestive, respiratory, and circulatory systems.
Scleroderma appears in various forms in different people as there are many types of the condition. Scleroderma is classified as either localized or systemic depending on the degree to which the skin is infected. The widespread form of the disease is considered systemic sclerosis.
Blacks are at slightly higher risk compared to whites. Women are three times more likely to develop the localized form of the disease than men. In fact, more than 80% of people with systemic scleroderma are women ages 30-50. Despite its serious implications, people with the disease normally live full lives though they may experience various degrees of discomfort and/or disability.
While the underlying cause of scleroderma remains unclear, the central feature of the disease is excessive production of collagen which is one of the sources from which skin is produced. Inappropriate amounts collagen causes the skin to be supple. However, when too much collagen accumulates it causes skin tissue to become thick and stiff, feeling almost leathery. Recent studies have focused on the connection between the immune system, the vascular system, and the growth of connective tissue which has suggested that the disease is not hereditary.
How is Scleroderma diagnosed?
Scleroderma is difficult to diagnose due to the fact that the symptoms and manifestations of the disease vary greatly between individuals and may often look like other diseases. The most common symptom is the tightening, hardening, and thickening of the skin on one’s arms, legs, hands, feet, and face. The skin is affected gradually during the early years of scleroderma’s onset until thickening stops at which time it can even recede.
Other symptoms of scleroderma are as follows:
One variation of systemic scleroderma known as CREST syndrome is recognized when five particular symptoms occur together. These symptoms include:
There is no one test that can directly identify scleroderma. However, your doctors can test for scleroderma in a few ways. One is testing antibodies in the blood, which may be positive in scleroderma although not diagnostic of the disease. Another test used to identify the earliest signs of scleroderma is capillary microscopy, which involves examining the small blood vessels known as capillaries at the nail fold. While this test is not a definitive indicator of scleroderma, it is helpful in arriving at a diagnosis when combined with other characteristic symptoms.
How is Scleroderma treated?
There is no known cure for scleroderma. Treatments for the disease aim to relieve the associated symptoms and reduce the risk of complications such as muscle and bone weakness, respiratory impairment, and/or digestive problems.
Some helpful treatments are as follows:
How can Scleroderma be prevented?
There is no known way to prevent scleroderma. Many cases of scleroderma are related to occupational exposures to silica dust found in mines and stone working settings. Solvents used in paint-thinners and removers can also trigger its onset. Although there is no ultimate prevention for scleroderma, various measures can be taken to prevent or lessen its symptoms including:
Authors: Derek Berberian, Syracuse University, Syracuse, New York
Marc Effron, M.D., Department of Physical Medicine and Rehabilitation, New York-Presbyterian Hospital, The University Hospital of Columbia and Cornell, New York City, New York