An Atypical Case of CREST Syndrome With Early Complete Clinical Manifestation

CREST syndrome, the limited cutaneous subtype of systemic sclerosis, is defined by five classic clinical features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, which also forms the initialism. While diagnosis requires the presence of only three criteria, the full expression of all five is uncommon and typically develops gradually over many years. We describe the case of a 44-year-old Spanish-speaking female patient who presented to a community-based health clinic. Her initial evaluation identified the recent onset of Raynaud's phenomenon, esophageal symptoms, sclerodactyly, and facial telangiectasia. Serology was notable for positive anti-centromere and antinuclear antibodies. One year later, she developed a painful mass on her right foot, ultimately identified as dystrophic calcification consistent with calcinosis. This case highlights a rare, rapidly progressive, and complete manifestation of CREST syndrome. This atypical presentation also underscores the importance of early recognition, multidisciplinary management, and careful attention to social determinants of health in patients with autoimmune disease.

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Publication Details

Journal
Cureus
Published
2025-06-21
DOI
https://doi.org/10.7759/cureus.86500
Primary Topic
Systemic Sclerosis and Related Diseases
Type
article
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0.00
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article

An Atypical Case of CREST Syndrome With Early Complete Clinical Manifestation

Didier Simon, G Schock, Raymond S. Weitzman, Elliot D. Weitzman
Cureus
Systemic Sclerosis and Related Diseases
article

An Atypical Case of CREST Syndrome With Early Complete Clinical Manifestation

Didier Simon, G Schock, Raymond S. Weitzman, Elliot D. Weitzman
article en

Abstract

CREST syndrome, the limited cutaneous subtype of systemic sclerosis, is defined by five classic clinical features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, which also forms the initialism. While diagnosis requires the presence of only three criteria, the full expression of all five is uncommon and typically develops gradually over many years. We describe the case of a 44-year-old Spanish-speaking female patient who presented to a community-based health clinic. Her initial evaluation identified the recent onset of Raynaud's phenomenon, esophageal symptoms, sclerodactyly, and facial telangiectasia. Serology was notable for positive anti-centromere and antinuclear antibodies. One year later, she developed a painful mass on her right foot, ultimately identified as dystrophic calcification consistent with calcinosis. This case highlights a rare, rapidly progressive, and complete manifestation of CREST syndrome. This atypical presentation also underscores the importance of early recognition, multidisciplinary management, and careful attention to social determinants of health in patients with autoimmune disease.

CureusVol. 17(6)
Michigan State University (US)
Openalex Percentile: Top 78%
Systemic Sclerosis and Related Diseases
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