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Granulomatosis with polyangiitis poses a significant diagnostic dilemma due its diverse presentations. Seemly isolated sites of disease, such as oral ulcers, may present to physicians working in primary care settings, the emergency room, and subspecialty fields as well as to dentists. Oral presentations are particularly challenging to identify and require a high index of suspicion and a detailed knowledge of the condition in order to diagnose and treat. We detail a case of granulomatosis with polyangiitis presenting as necrotizing gingivitis, one of the first of its kind to be reported.    Case presentation  An otherwise healthy 32-year-old, Caucasian woman presented to various physicians with progressive, painful oral ulcers. Following consultations with multiple primary care physicians and subspecialties, an initial diagnosis of severe infectious necrotizing gingivitis was made resulting in combination antibiotic treatment as well as surgical debridement involving extraction of all maxillary and three mandibular teeth. With the discovery of a positive cytoplasmic anti-neutrophil cytoplasmic antibody and a constellation of associated systemic symptoms, our patient was subsequently diagnosed with granulomatosis with polyangiitis. The treatment regimen of rituximab and methylprednisone was chosen in consideration of our patient’s desire for future fertility and has been successful in inducing and maintaining remission.    Conclusions  Following the case presentation, we review the current literature regarding granulomatosis with polyangiitis presentation, diagnosis and treatment. In discussing features of granulomatosis with polyangiitis presentation, diagnostic tests, and important new treatment options, we seek to enable physicians of all specialties to better recognize and begin appropriate treatment for this complex condition.

Granulomatosis with polyangiitis (Wegener’s) as a necrotizing gingivitis mimic: a case report