Circumferential Eschar-Covered Necrotic Leg Ulcers in a Case of Granulomatous Vasculitis: Looking Beyond ANCA Serology

Cutaneous vasculitis, either as a single organ or part of systemic vasculitis, can take various forms. Granulomatosis with polyangiitis (GPA) is characterized by necrotizing granulomatous inflammation in the respiratory tract and vasculitis affecting small- to medium-sized blood vessels. Skin-limited GPA, an uncommon presentation, poses diagnostic challenges and may result in delayed diagnosis. We describe a 32-year-old man with painful ulcers and black eschars on both lower limbs, evolving from purpura. Despite lacking typical anti neutrophil cytoplasmic antibodies and systemic involvement, histopathology revealed granulomatous vasculitis. Treatment with prednisolone and methotrexate led to complete symptom resolution within 8 months. Skin-limited GPA, often without anti neutrophil cytoplasmic antibody positivity, warrants clinical suspicion, early intervention, and increased awareness to enhance patient outcomes.