Diseases Home > CREST Syndrome

CREST syndrome -- which stands for calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias -- is a common name that healthcare providers give to limited scleroderma. Scleroderma is a disease characterized by an overproduction of collagen. It can affect nearly every part of the body, and symptoms may begin gradually and then worsen.

What Is CREST Syndrome?

Scleroderma is an activation of immune cells, which produces scar tissue in the skin, internal organs, and small blood vessels. Women are three times more likely than men to develop scleroderma. Although scleroderma appears to be more common among African American women than other races, a woman's risk of developing scleroderma is 15 times greater during her childbearing years.
 
The two types of scleroderma are:
 
 
Localized scleroderma affects only certain parts of the body, while systemic scleroderma can affect the whole body. In addition, each type of scleroderma has subtypes. One of the subtypes of systemic scleroderma is limited scleroderma, which doctors often refer to as CREST syndrome.

(Click Types of Scleroderma for more information on the different types.)
  

How Did CREST Syndrome Get Its Name?

People with systemic scleroderma often have all or some of the symptoms that doctors refer to as CREST syndrome. CREST stands for the following:
 
 

Understanding Limited Scleroderma

Some healthcare providers refer to limited scleroderma as CREST syndrome because of the predominance of CREST symptoms.
 
Limited scleroderma typically comes on gradually and affects the skin only in certain areas, which include:
 
  • Fingers
  • Hands
  • Face
  • Lower arms
  • Legs.
 
Many people with CREST syndrome have Raynaud's phenomenon for years before skin thickening starts. Other people with CREST syndrome start out with skin problems over much of the body, which improve over time, leaving only the face and hands with tight, thickened skin. In CREST syndrome, telangiectasias and calcinosis often follow.
 
Written by/reviewed by:
Last reviewed by: Arthur Schoenstadt, MD
Last updated/reviewed:
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