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CREST

Evidence: Antibody

Names

CREST
A subtype of Scleroderma

Description

CREST is a subtype of scleroderma, a connective tissue disease characterized by changes in the skin, blood vessels, skeletal muscles, and internal organs. CREST is the acronym for the clinical features seen in individuals with the disease:                        

  • C - Calcinosis: calcium deposits in the connective tissues

  • R - Raynaud's phenomenon: where the hands and feet turn white and cold and then blue, in response to cold or anxiety.

  • E - Esophageal dysfunction: where a person has difficulty swallowing

  • S - Sclerodactyly: thick and tight skin on the fingers, caused by an excess of collagen deposits within skin layers.

  • T - Telangiectasia: small red spots on the hands and face that are caused by the swelling of tiny blood vessels.

To be diagnosed with CREST, you must have 3 out of the 5 symptoms.

Patient Groups

Prevalence

Typical Age of onset

40-50 years

Symptoms

  • Abnormal finger tip shape or thickness of skin (sclerodactly)

  • Firm, pimple-like bumps or nodules on the skin that are white or yellow (calcinosis)

  • Swelling caused by excess fluid trapped in your body’s tissues (edema)

  • Periods of abdominal discomfort including nausea, emptiness, tightness, churning, butterflies, malaise, pain and hunger. Sensation may rise to chest or throat. May be preceded by an “aura” that involves impaired vision or other perceptions (epigastric aura)

  • Lack of skin elasticity

  • Nausea

  • Vomiting

  • Stiff skin

  • Difficulty with swallowing (dysphagia)

  • difficult or labored breathing (dyspnea)

  • Threadlike red lines or patterns on the lips and skin, sometimes called “spider veins” (telangiectasia)

  • Skin ulcers