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CREST syndrome
See also in: Nail and Distal Digit
Other Resources UpToDate PubMed

CREST syndrome

See also in: Nail and Distal Digit
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

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Telangiectasias on face and lips resemble Hereditary hemorrhagic telangiectasia.
  • Porphyria cutanea tarda can have sclerodermatous face and hand lesions.
  • Calcinosis cutis can occur in Dermatomyositis (especially the Juvenile dermatomyositis), and it may have prominent red and purple atrophic lesions that can be confused with SSc.
  • Chronic graft-versus-host disease should be clear from history of a transplant.
  • Scleromyxedema often has a monoclonal gammopathy.
  • CREST syndrome may also be confused with Scleredema associated with diabetes mellitus or a streptococcal infection.
  • Nephrogenic systemic fibrosis results in diffuse fibrosis but lacks the other features of SSc. This most frequently occurs in individuals with renal disease who were given gadolinium contrast.
  • Eosinophilic fasciitis presents with distal extremity sclerosis but lacks other features of SSc.

Best Tests

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Management Pearls

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Therapy

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References

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Last Reviewed:04/11/2019
Last Updated:04/11/2019
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CREST syndrome
See also in: Nail and Distal Digit
A medical illustration showing key findings of CREST syndrome : Joint contractures, Nail fold telangiectasia, Sclerodactyly, Telangiectasia, Dysphagia, Dyspepsia
Clinical image of CREST syndrome - imageId=64099. Click to open in gallery.  caption: 'Violaceous, telangiectatic macules on the fingers.'
Violaceous, telangiectatic macules on the fingers.
Copyright © 2024 VisualDx®. All rights reserved.