Five Key Features of CREST Syndrome
The five key features of CREST syndrome are calcium deposits in the subcutaneous layer of skin, extreme sensitivity to cold and stress, skin that tightens and becomes discolored around fingers and toes, and difficulty swallowing. People with this syndrome may also develop interstitial lung disease and pulmonary arterial hypertension. It is a relatively rare disease. However, it can be life-threatening. In severe cases, the patient may develop severe complications, including scleroderma, pulmonary arterial hypertension, and scleroderma renal crisis.
Raynaud's phenomenon
Raynaud's phenomenon is an overreaction of the body to cold temperature and stress. This causes abnormalities in the blood vessels, which can lead to numbness and pain in the fingers. It can be life-threatening if it is not diagnosed and treated quickly.
Esophageal dysfunction
Esophageal dysfunction can be a symptom of CREST syndrome, a genetic disorder characterized by scarring of the esophagus (tube leading from the mouth to the stomach). In addition to dysphagia, this condition can lead to calcinosis, Raynaud's phenomenon, and sclerodactyly. Other symptoms include a tight skin appearance and red patches known as telangiectasias.
Calcinosis
Calcinosis in CREST syndrome is a rare, chronic disorder characterized by calcification of the connective tissues. This condition often results from a combination of systemic physiological deficits and immune dysfunction. This multifactorial etiology makes it difficult to diagnose and treat. Fortunately, ablation of calcific deposits may offer some hope.
Skin thickening
CREST syndrome, or skin thickening due to systemic sclerosis, affects the skin. People with this condition will often experience skin thickening, often restricted to the hands, forearms and face. This condition also results in red spots called telangiectasias, which are caused by widened blood vessels.
Joint stiffness
CREST syndrome is an uncommon form of scleroderma characterized by a constellation of symptoms, including calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and Telangiectasia. The syndrome can be difficult to diagnose and treat. It may also lead to dry skin and joint stiffness. Treatment options include moisturizers, antacids, and blood pressure pills.
Telangiectasia
CREST syndrome and Telangiectasia are related to autoimmune diseases. Both of these conditions are characterized by abnormalities of the blood and skin, which are associated with autoimmune inflammation. Serologic studies can confirm the diagnosis. Treatment is similar to that of systemic sclerosis. Treatment is usually aimed at relieving symptoms and preventing underlying disease development.
