CREST Syndrome (Limited Scleroderma)

CREST Syndrome (Limited Scleroderma)

Blake St. Clair

Author Bio -

Key Points:

*Dermatologic manifestations include hard calcium deposits on the fingers called calcinosis cutis, Raynaud’s discoloration of the fingers, sclerodactyly or tightening of the skin over the hands, and clusters of dilated blood vessels called telangiectasia

CREST Syndrome is a limited variant of scleroderma, a chronic connective tissue disease in which a dense protein, collagen, is deposited inappropriately by cells in the skin and throughout the body.  CREST is an acronym for the primary sites of involvement.  C is for Calcinosis cutis, which is calcium deposition that appears as small, hard nodules on the fingers or elsewhere.  R is for Raynaud’s, decreased blood flow to the fingers that manifests as white or blue discoloration upon cold exposure.  E is for Esophageal dysmotility, or improper functioning of the food pipe during swallowing. S is for Sclerodactyly, a characteristic tightening of the skin of the fingers and hands. T is for Telangiectasia, or tiny, torturous blood vessels visible in clusters on the skin.

CREST syndrome is also called “limited scleroderma” because unlike the “diffuse” variant of scleroderma, there is no involvement of the heart, lungs, or kidneys.  CREST syndrome is more common in women than in men, and typically has its onset in middle age.

Differential Diagnosis

Diffuse scleroderma

Diagnosis

*History and physical exam are key to diagnosis

*Anti-centromere antibodies are very specific for CREST syndrome

The diagnosis of CREST syndrome is largely based on history and typical exam findings.  In addition, the presence of Anti-centromere antibodies in blood is highly specific for this variant of scleroderma.  Anti-nuclear antibodies are almost always elevated but this blood marker is elevated in several other conditions.  Studies designed to look at the ability of the esophagus, the food pipe, to move food to the stomach may also be of use.

Treatment

*There is no definitive cure

*Treatment is based on controlling symptoms/dermatologic manifestations

Calcinosis cutis may be treated with various medical interventions or be surgically removed.  Raynaud’s may be managed by ensuring that the fingers stay warm with the use of gloves and cold avoidance.  In addition, smoking cessation in a current smoker and calcium channel blockers may be used to treat Raynaud.  Sclerodacyly is an irreversible process of skin tightening, but associated itching may be relieved with topical steroids.  Telangiectasias are of cosmetic importance only but may be treated with laser ablation.

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