Granulomatosis with polyangiitis (GPA) is a rare multisystem autoimmune disease of unknown etiology involving small to medium vessels. It is one of the antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis disorders. Its hallmark features include necrotizing granulomatous inflammation and pauci-immune vasculitis. GPA with aseptic abscess is an even more uncommon presentation. This is the case of a 56-year-old female who presented to our outpatient department (OPD) with cough, breathlessness, right-sided chest pain, multiple cutaneous abscesses, and ulcers in lower extremities. Radiology evaluation showed bilateral nodules and cavitary lesions. c-ANCA was found to be positive. Skin biopsy was consistent with features of vasculitis. She was diagnosed with GPA with aseptic abscess syndrome and treated effectively with rituximab. The combination of GPA with aseptic abscess is a rare clinical entity. The mainstay of treatment is anti-inflammatory drugs.
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