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

## TL;DR

A rare case of CREST syndrome led to severe peripheral vascular issues, including auto-amputation, highlighting the need for early diagnosis and treatment.

## Contribution

This case report presents an unusual progression of CREST syndrome with critical limb ischemia and auto-amputation.

## Key findings

- A 48-year-old female with CREST syndrome developed progressive ischemia and auto-amputation of fingers and toes.
- CT angiography showed segmental and progressive obstruction of peripheral arteries.
- Immunomodulatory therapy stabilized the patient's condition and prevented further complications.

## 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.

CREST syndrome, a limited systemic sclerosis variant, may rarely present with progressive peripheral artery disease causing critical limb ischemia and auto‐amputation. Absence of traditional cardiovascular risk factors should raise suspicion for autoimmune vasculopathy. Early recognition, vascular imaging, immunomodulatory therapy, and multidisciplinary management are essential to prevent irreversible complications and limb loss.