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CREST syndrome
See also in: Nail and Distal Digit
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CREST syndrome

See also in: Nail and Distal Digit
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Contributors: Jeffrey M. Cohen MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

CREST syndrome is a subtype of limited cutaneous systemic sclerosis (lcSSc). The acronym outlines the most common features of this rare subtype of lcSSc: Calcinosis cutis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. Patients may complain of dysphagia, chronic heartburn, dyspnea, joint contractures, and diminished exercise capacity. Patients with CREST syndrome, as with all forms of lcSSc, have a lower frequency of cardiac and renal involvement and a more prolonged survival when compared to patients with diffuse cutaneous systemic sclerosis (dcSSc), the more generalized form of systemic sclerosis (SSc). Given that many patients with lcSSc do not display all 5 features of CREST syndrome, the lcSSc terminology is favored.

Patients with lcSSc can be differentiated from those with dcSSc by the limited skin involvement to distal extremities. The anticentromere antibody, while only present in approximately 40% of individuals with lcSSc, is a laboratory test used to differentiate the two entities.

There is also a rare subtype of SSc that lacks skin sclerosis but has Raynaud phenomenon, nail fold capillary changes, internal organ involvement, and positive serologies called SSc sine scleroderma that has a similar trajectory to lcSSc. 

Codes

ICD10CM:
M34.1 – CR(E)ST syndrome

SNOMEDCT:
31848007 – CREST syndrome

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Last Reviewed: 04/12/2019
Last Updated: 04/12/2019
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CREST syndrome
See also in: Nail and Distal Digit
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CREST syndrome : Joint contractures, Nail fold telangiectasia, Sclerodactyly, Telangiectasia, Dysphagia, Dyspepsia
Clinical image of CREST syndrome
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