# Skin, Heart, and CNS Involvement in Granulomatosis With Polyangiitis: A Case Report

## TL;DR

A 60-year-old man with skin, heart, and brain symptoms was diagnosed with a rare form of vasculitis, highlighting the difficulty in distinguishing it from infections.

## Contribution

This case report highlights the rare co-occurrence of skin, heart, and CNS involvement in ANCA-associated vasculitis.

## Key findings

- The patient showed pauci-immune segmental vasculitis consistent with ANCA-associated glomerulonephritis.
- The case demonstrates the diagnostic challenge of differentiating vasculitis from infective endocarditis.
- The patient improved with high-dose steroid and immunomodulator therapy.

## Abstract

We discuss the case of a 60-year-old male who presented with ankle pain, a necrotic rash, and progressive weakness in both lower limbs and the right upper limb. An infectious workup of the skin lesions came back negative. Additionally, his kidney function tests indicated an acute kidney injury. This prompted investigations for vasculitis etiologies, which revealed a positive cytoplasmic antineutrophil cytoplasmic autoantibody (c-ANCA). His neurological deficits were also investigated, and imaging suggested embolic infarcts. Cardiac imaging showed valve vegetations and blood culture showed a lack of growth suggestive of a noninfective nature of these lesions. Based on all these findings, a kidney biopsy was obtained and demonstrated pauci-immune segmental vasculitis consistent with ANCA-associated glomerulonephritis. As such, the patient showed improvement with heavy pulse steroid and immunomodulator therapy. Although skin, heart, and CNS involvement have been previously reported with ANCA-associated vasculitis, it is rare, especially together, and can prove a diagnostic challenge. Therefore, it is important to consider vasculitis etiology in patients presenting similarly. In addition, this case highlights the overlapping clinical picture between infective endocarditis and vasculitis with valvular involvement, making differentiation between the two challenging.