Granulomatosis with polyangitis, formerly known as Wegener's granulomatosis, is a multi-system vasculitis that has a variable clinical presentation. Although uncommon, cutaneous symptoms can be the initial presenting symptom of granulomatosis with polyangitis. We present an unusual case of pyoderma gangrenosum followed by a diagnosis of granulomatosis with polyangitis. We also provide a review of current literature on therapeutic options.
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