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.


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.


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.


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

CREST syndrome

ADAM Medical Encyclopedia

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