The word "scleroderma" means "hardening of the skin" and refers to one of the possible physical effects of the disease.
Localized scleroderma is referred to as morphea or linear scleroderma and does not affect the internal organs. People who develop localized scleroderma usually do not develop generalized scleroderma
Approximately 300,000 people in the United States have scleroderma. Women are affected three times as often as men. Although the disease can develop at any age, it most frequently begins past middle age. It is most frequently a chronic and often progressive illness.
In someone suffering with scleroderma, collagen (a protein manufactured by the connective tissues of the body) is produced in excess. This over-production of collagen can be likened to the process of scarring, which is the way the body heals a wound. For persons with scleroderma, the scarring gets out of hand. The production of collagen is unregulated and therefore, abnormal. Consequently, excess collagen is deposited in various organs and/or tissues of the body, especially in layers of the skin.
It is important to stress that the symptoms of scleroderma vary greatly from person to person. It is as though each person with scleroderma has his or her own version of the disease. Although scleroderma can indeed be serious, most people are able to live a normal life span with varying degrees of discomfort and/or disability.
There are two forms of scleroderma: localized and generalized (also called systemic sclerosis).
Localized scleroderma affects mainly the skin. It can affect skin in different areas of the body. It may also affect muscles and bone, but it does not affect internal organs. This form is usually not as severe as generalized scleroderma. People who develop localized scleroderma usually do not develop generalized scleroderma.
There are two types of localized scleroderma: morphea and linear. Early morphea scleroderma has an inflammatory stage, followed by one or more slowly enlarging patches or plaques. These plaques are most commonly oval in shape and vary in size. They have an ivory/yellow center and are surrounded by a violet colored area. The violet color signifies that the scleroderma is in a state of activity. The plaques feel firm and hard, but are not deeply bound down. They may be depressed or slightly elevated and are seen more often on the trunk, but may also occur on the face and extremities.
Generalized morphea may involve almost the entire skin surface.
An uncommon form of morphea is the guttate variety. It is characterized by multiple, small, chalk-white spots which vary in size from 1 to 10mm in diameter. The violet-colored line may surround all or some of the spots and in cases of long duration the line may be brown or grayish. Guttate morphea primarily involves the chest, neck and shoulders, and only occasionally other parts of the body. Localized morphea may last from a few months to many years. However, a large proportion of morphea patients improve spontaneously.
The cause of scleroderma is unknown. It is not contagious. It is not inherited or passed on from one generation to the next, except in rare circumstances. We do know that in scleroderma, the body produces too much of a protein called collagen. This excess collagen is deposited in the skin and in body organs, which causes thickening and hardening of the skin and affects the function of internal organs.
What Questions to ask Your Doctor About Morphea Scleroderma?
- What tests are used to diagnose which types of Scleroderma?
· What type of Scleroderma do I have?
· What medications will you prescribe? What are the side effects?
· How long after medication is initiated will there be relief or see a decrease in the Scleroderma?
· Is there anything that aggravates the condition?
· Is there anything that suppresses or stimulates the production of collagen?
· Are there any signs or symptoms that may appear in the future that need to be reported immediately?
· What is recommended to protect the skin? The hands?
· What exercise program will be recommended?
· Is there a support group in the area that could help cope with this condition?
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