Work

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

Year: 2026

Type: article

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.

Source: Clinical Case Reports

DOI: 10.1002/ccr3.71815

Authors: Sakshi Kumari, Fathimathul Henna, Umama Alam, Fazia Khattak, Kamil Ahmad Kamil

Institutions: Dubai Medical College, Khyber Medical College

Language: English

Topic: Systemic Sclerosis and Related Diseases

Subfield: Pathology and Forensic Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $1,820