Alternative Names
CREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Morphea, linear
Definition of Scleroderma
Scleroderma is a widespread connective tissue disease that involves changes in the skin, blood vessels, muscles, and internal organs.
Causes, incidence, and risk factors
The cause of scleroderma is unknown. People with this condition have a buildup of a substance called collagen in the skin and other organs. This buildup leads to the symptoms of the disease.
Symptoms
Localized scleroderma usually affects only the skin on the hands and face. Its course is very slow, and it rarely, if ever, spreads throughout the body (becomes systemic) or causes serious complications.
Signs and tests
Examination of the skin may show tightness, thickening, and hardening.
Treatment
There is still no treatment for the disease process of scleroderma. However, certain drugs and treatments help combat the complications.
Expectations (prognosis)
In most patients, the disease slowly gets worse. People who only have skin symptoms have a better outlook. Death may occur from gastrointestinal, heart, kidney, or lung complications.
Review
Mark James Borigini, MD, Rheumatologist in the Washington, DC Metro area. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. – 2/7/2010
