# Ischemic Necrosis of Bilateral Second Toes in the Absence of Macrovascular Disease or Primary Vasculitis: A Unique Case of Systemic Sclerosis Sine Scleroderma (ssSSc)

## TL;DR

A 51-year-old woman with no macrovascular disease or vasculitis developed toe ischemia, later diagnosed with systemic sclerosis sine scleroderma, highlighting the importance of considering rare autoimmune conditions in unexplained vascular cases.

## Contribution

This case report presents a rare manifestation of systemic sclerosis sine scleroderma with toe ischemia, emphasizing its diagnostic challenges and management.

## Key findings

- The patient had ischemic necrosis of bilateral second toes without macrovascular disease or vasculitis.
- Serologic testing showed high-titer centromere-pattern ANA, and histopathology revealed thrombotic vasculopathy.
- Conservative treatment led to complete epithelialization within eight weeks.

## Abstract

Systemic sclerosis sine scleroderma (ssSSc) is an uncommon subset of systemic sclerosis, characterized by internal organ involvement and autoimmune serologies in the absence of skin thickening. Diagnosis is frequently delayed due to the lack of cutaneous findings, particularly when vascular manifestations precede classical rheumatologic features.

We report a compelling case of a 51-year-old woman who developed bilateral plantar second-toe ischemic necrosis despite having no diabetes, macrovascular disease, or known vasculitis. Extensive vascular imaging, including arterial duplex, ankle-brachial indices (ABIs), and toe photoplethysmography (PPG), was unremarkable. Serologic testing revealed a high-titer, centromere-pattern antinuclear antibody (ANA), and histopathology demonstrated thrombotic vasculopathy without features diagnostic of immune complex vasculitis. Nailfold capillaroscopy showed dilated capillary loops, and rheumatologic evaluation confirmed the diagnosis of ssSSc. The patient was treated conservatively with aspirin, nifedipine, and local wound care, resulting in complete epithelialization within eight weeks. This case highlights the importance of considering ssSSc in the differential diagnosis of unexplained digital ischemia - particularly in the lower extremities - and underscores the value of multidisciplinary collaboration in identifying rare autoimmune vascular syndromes presenting with limb-threatening ischemia.