Sweet's syndrome as a presenting symptom of granulomatosis with polyangiitis

A patient presented with fatigue, weight loss, haemoptysis, bullous rash and spiculated pulmonary nodules. Skin biopsy was consistent with Sweet's syndrome (SS). Pertinent labs included a positive c- anti-neutrophil cytoplasmic antibody (ANCA) and positive proteinase 3 (PR3) antibody. Additional work-up included a lung biopsy which revealed multinucleated giant cells and repeat skin biopsy with small and medium vasculitis, leading to a diagnosis of granulomatosis with polyangiitis (GPA). SS is a neutrophilic dermatosis that often presents with malignancy, drugs or infection. SS has rarely been described in association with ANCA-vasculitis, with few reports of SS as a presenting symptom. Patients presenting with SS and additional features concerning for ANCA-vasculitis may benefit from further workup for vasculitis given the high morbidity and mortality associated with the disease.