What Is CREST Syndrome?

D. Jeffress

CREST syndrome is a potentially serious disorder that causes damage to skin and soft connective tissues in the body. The condition's name is an acronym that stands for its major symptoms: calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. People who have CREST syndrome might experience acute or chronic episodes of hand and foot pain, skin lesions, joint stiffness, fatigue, and a number of other complications. Treatment may consist of daily medication use, lifestyle changes, and occasionally surgery to remove sections of badly damaged skin or soft tissue.

Inflammation of the esophagus may cause difficulties swallowing.
Inflammation of the esophagus may cause difficulties swallowing.

The causes of CREST syndrome are not well understood. Some people have family histories of the disease, suggesting a genetic component, but the majority of patients appear to develop symptoms spontaneously. Many studies have linked the condition with environmental factors such as long-term exposure to industrial toxins, glues, and formaldehyde. This syndrome is more common in females than in males, and the average age of onset is about 40.

Patients may experience severe digestive issues if telangiectasia, the final element of CREST syndrome, is present.
Patients may experience severe digestive issues if telangiectasia, the final element of CREST syndrome, is present.

The first component of CREST syndrome, calcinosis, refers to the buildup of hard calcium deposits in soft tissue. It usually affects the fingers, resulting in pain, tenderness, and occasionally causing skin ulcers to develop. Raynaud phenomenon is characterized by skin changes in the fingers and toes in response to stress or temperature fluctuations. The skin at the ends of the digits can quickly turn very pale or blue and become numb. Esophageal dysmotility refers to inflammation and partial paralysis of the esophagus, leading to difficulties swallowing and speaking.

Cold or numbness in the toes may be a symptom of Raynaud's phenomenon.
Cold or numbness in the toes may be a symptom of Raynaud's phenomenon.

Sclerodactyly is a complication in the fingers and toes that causes the skin to become thick and tough, which can reduce joint mobility. The final element of the syndrome, telangiectasia, is a blood vessel disorder that causes bleeding and inflammation underneath the skin or in the intestines. Patients might have painful open skin lesions or severe digestive issues if telangiectasia is present. Not all patients who are diagnosed with CREST syndrome present with signs of all five component disorders; in fact, most people only have two or three symptoms.

Hand pain and joint stiffness are possible symptoms of someone with CREST syndrome.
Hand pain and joint stiffness are possible symptoms of someone with CREST syndrome.

A doctor who suspects CREST syndrome typically performs several diagnostic tests. Blood and skin samples are analyzed in a laboratory, and imaging scans are taken of the fingers and toes. Specialists carefully document the type and severity of each symptom in order to make the best treatment decisions.

Sclerodactyly is a complication which causes the skin on the fingers and toes to become thick and tough and reduces joint mobility.
Sclerodactyly is a complication which causes the skin on the fingers and toes to become thick and tough and reduces joint mobility.

Most patients are placed on daily medication schedules consisting of anti-inflammatory drugs, immune system suppressants, and blood pressure regulators. A person with esophageal dysmotility may need to adopt a specialized diet plan, and sclerodactyly might require light therapy to loosen and thin hard skin. In addition, physical therapy helps many people rebuild flexibility in sore joints. With ongoing treatment, most patients are able to effectively manage their conditions and enjoy daily life.

Esophageal dysmotility refers to inflammation and partial paralysis of the esophagus, leading to difficulties swallowing and speaking.
Esophageal dysmotility refers to inflammation and partial paralysis of the esophagus, leading to difficulties swallowing and speaking.
Blood pressure regulators may be prescribed to someone with CREST syndrome.
Blood pressure regulators may be prescribed to someone with CREST syndrome.
Some people may have a family history of CREST syndrome, which suggests a genetic component to the disease.
Some people may have a family history of CREST syndrome, which suggests a genetic component to the disease.

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Discussion Comments

anon154466

Is stretching of the shin and a painful ankle part of scleroderma. What is the medical help for this problem as I find it difficult to walk. --xhku

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