Treatment of severe perinuclear antineutrophil cytoplasmic antibody–associated vasculitis with efgartigimod

Antineutrophilic cytoplasmic antibody–associated vasculitis (AAV) is a heterogenous group of rare autoimmune disorders characterized by inflammation of small and medium-sized arteries. This inflammation can manifest in multiple organ systems including the kidneys, lungs, and skin. Cutaneous involvement of AAV most commonly presents with palpable purpura but can also cause a variety of dermatologic findings include vesicles, blisters, and necrotic-ulcerative lesions.1 Cutaneous manifestations can be severe and are associated with life-threatening systemic manifestations including alveolar hemorrhage and glomerulonephritis.