CREST syndrome causes damage to the skin and soft connective tissues, with symptoms including calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. Treatment may involve medication, lifestyle changes, and surgery. The causes are not well understood, but environmental factors may play a role. Diagnosis involves diagnostic tests and careful documentation of symptoms. Treatment options include medication, dietary changes, light therapy, and physical therapy.
CREST syndrome is a potentially serious disease that causes damage to the skin and soft connective tissues of the body. The name of the condition is an acronym that stands for its main symptoms: calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. People who have CREST syndrome might experience acute or chronic episodes of hand and foot pain, skin breakdown, joint stiffness, fatigue, and a host of other complications. Treatment may consist of daily use of medications, lifestyle changes, and occasionally surgery to remove sections of severely damaged skin or soft tissue.
The causes of CREST syndrome are not well understood. Some people have family histories of the disease, which suggests a genetic component, but most patients appear to develop symptoms spontaneously. Many studies have linked the condition to 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 mean age of onset is around 40 years.
The first component of CREST syndrome, calcinosis, refers to the accumulation of hard deposits of calcium in the soft tissues. It usually affects the fingers, causing pain, soreness, and occasionally causing skin ulcers to develop. Raynaud’s phenomenon is characterized by changes in the skin of the fingers and toes in response to stress or changes in temperature. The skin on the ends of the fingers can quickly turn very pale or blue and become numb. Esophageal dysmotility refers to inflammation and partial paralysis of the esophagus, leading to difficulty swallowing and speaking.
Sclerodactyly is a complication of the fingers and toes that causes the skin to become thick and hard, which can reduce joint mobility. The final element of the syndrome, telangiectasia, is a blood vessel disease that causes bleeding and inflammation under the skin or in the intestines. Patients might have painful open skin lesions or severe digestive problems if telangiectasia is present. Not all patients diagnosed with CREST syndrome have signs of all five component disorders; in fact, most people have only two or three symptoms.
A doctor who suspects CREST syndrome typically does several diagnostic tests. Blood and skin samples are analyzed in the laboratory, and image scans of fingers and toes are taken. Specialists carefully document the type and severity of each symptom in order to make the best treatment decisions.
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 dietary plan, and sclerodactyly may require light therapy to loosen and thin the tough skin. Additionally, physical therapy helps many people rebuild flexibility in sore joints. With ongoing treatment, most patients are able to manage their conditions effectively and enjoy their daily lives.
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