Sweet syndrome in a patient with granulomatosis with polyangiitis

Sweet syndrome (acute febrile neutrophilic dermatosis) is rarely known to be associated with Granulomatosis with polyangiitis (GPA), a necrotizing vasculitis of small- and medium-sized blood vessels predominantly affecting respiratory tract, and kidneys. A 66-year-old woman with history of rhinosinusitis, adult onset asthma was seen in medicine clinic for recurrent epistaxis, paranasal sinus congestion, wheezing, dyspnea for 2 years despite maximal medical therapy and sinus surgery. Labs were significant for acute kidney injury (AKI); positive C-anti-neutrophil cytoplasmic antibody (1:5,120); high ESR, CRP, and anti-PR3 (anti–proteinase 3); and microscopic hematuria.