Oral ulceration as a revealing sign of granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA), formerly called Wegener's granulomatosis, is a rare systemic disease of unknown etiology that can affect all areas of the body, including the oral cavity. Typical oral manifestations present as nonspecific erosive/ulcerative lesions of the oral mucosa or appear with hyperplastic gingivitis, termed “strawberry gingivitis”. We report here a case of cheek ulceration as the first sign of recurrence of GPA in the absence of oral manifestations in the primary disease. A 37-year-old woman was referred to our oral pathology and surgery department for oral ulcerations. The patient had already been diagnosed with GOODPASTURE syndrome (pneumorenal syndrome) two years previously and treated with cyclophosphamide and Azathioprine. Its relapse and the appearance of oral manifestations made it possible to readjust the diagnosis and the treatment.