Abstract
Sweet syndrome, also known as acute febrile neutrophilic dermatosis, is a neutrophilic dermatosis characterized by the presence of painful erythematous plaques and nodules with a sterile neutrophilic infiltrate on histology. However, the clinical manifestations of sweet syndrome can vary widely. Additionally, Sweet syndrome is often associated with underlying systemic diseases. In this case report, we present the case of a 72-year-old female who developed bullous sweet syndrome with oral involvement following a blood transfusion. Serology revealed an elevated pANCA level and systemic manifestations consistent with granulomatosis with polyangiitis. There have been approximately 50 reported cases of concurrent neutrophilic dermatoses occurring with ANCA-associated vasculitis. Relapse is common in these patients, with all reported cases involving the ears, nose, and throat (ENT) region. Ultimately, the concomitant presentation of ANCA-associated vasculitis and neutrophilic dermatoses is rare. Dermatologists should be aware that testing for ANCA in cases of neutrophilic dermatoses is appropriate, and if positive, close monitoring for signs of systemic vasculitis is warranted.
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