Beyond Raynaud's: Atypical Peripheral Vascular Manifestations in a Case of <scp>CREST</scp> Syndrome

Peripheral vascular disease (PVD) is a rare but severe symptom of CREST syndrome, which itself is a limited cutaneous sclerosis. Even though Raynaud's phenomenon is the gold standard of CREST, the development of critical limb ischemia and self-amputation is rare. We report a case of a 48-year-old female with CREST syndrome who presented with progressive ischemia in both upper and lower limbs, leading to spontaneous auto-amputation of multiple fingers/toes. The patient exhibited sclerodactyly, dry gangrene, delayed capillary refill, and tactile deficits in peripheral pulses. ANA and anti-Scl 70 antibody were positive. CT angiography demonstrated segmental and progressive obstruction of multifocal peripheral arteries. The patient was operated on for amputation of necrotic digits and was initiated on immunomodulatory therapy. Following treatment, monitoring revealed stabilization without further progression. This case highlights the possible seriousness of vascular complications in CREST syndrome. Prompt diagnosis and treatment are necessary to prevent irreversible ischemic damage.

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Journal
Clinical Case Reports
Published
2026-01-01
DOI
https://doi.org/10.1002/ccr3.71815
Primary Topic
Systemic Sclerosis and Related Diseases
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article
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article

Beyond Raynaud's: Atypical Peripheral Vascular Manifestations in a Case of CREST Syndrome

Shweta Kumari, Umama Alam, Fathimathul Henna, Kamil Ahmad Kamil et al.
Clinical Case Reports
Systemic Sclerosis and Related Diseases
article

Beyond Raynaud's: Atypical Peripheral Vascular Manifestations in a Case of CREST Syndrome

Shweta Kumari, Umama Alam, Fathimathul Henna, Kamil Ahmad Kamil, Fazia Khattak
article en

Abstract

Peripheral vascular disease (PVD) is a rare but severe symptom of CREST syndrome, which itself is a limited cutaneous sclerosis. Even though Raynaud's phenomenon is the gold standard of CREST, the development of critical limb ischemia and self-amputation is rare. We report a case of a 48-year-old female with CREST syndrome who presented with progressive ischemia in both upper and lower limbs, leading to spontaneous auto-amputation of multiple fingers/toes. The patient exhibited sclerodactyly, dry gangrene, delayed capillary refill, and tactile deficits in peripheral pulses. ANA and anti-Scl 70 antibody were positive. CT angiography demonstrated segmental and progressive obstruction of multifocal peripheral arteries. The patient was operated on for amputation of necrotic digits and was initiated on immunomodulatory therapy. Following treatment, monitoring revealed stabilization without further progression. This case highlights the possible seriousness of vascular complications in CREST syndrome. Prompt diagnosis and treatment are necessary to prevent irreversible ischemic damage.

Clinical Case ReportsVol. 14(1)
Khyber Medical College (PK), Kandahar University (AF), Dubai Medical College (AE), Institute of Medical Sciences (IN)
Gender equality
Openalex Percentile: Top 93%
Systemic Sclerosis and Related Diseases
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Beyond Raynaud's: Atypical Peripheral Vascular Manifestations in a Case of <scp>CREST</scp> Syndrome — Shweta Kumari, Umama Alam, et al. · Clinical Case Reports (2026) | TGRS Research Map | TGRS