Childhood-Onset Granulomatosis With Polyangiitis as a Palatal Defect: A Case Report

Granulomatosis with polyangiitis (GPA) is a necrotizing systemic vasculitis of small and medium-sized vessels with renal and sinopulmonary involvement. Its symptoms include chronic sinusitis, recurrent pneumonia, glomerulonephritis, constitutional symptoms, and skin manifestations with a typical onset in the fourth to sixth decade of life. We present a rare case of GPA in a 16-year-old female who presented with facial numbness and nasal regurgitation via a palatal defect. The patient reported a several-month history of recurrent epistaxis and chronic nasal congestion accompanied by several weeks of night sweats, lower right-sided facial numbness and pain, nasal regurgitation of food and liquids, and a 30-pound weight loss. A physical exam found a 3-cm defect on the right side of her palate. CT of the sinuses showed significant sinonasal destruction and petrous apicitis. GPA was confirmed via pathognomonic chest X-ray findings and biopsy results. The patient was treated with maxillary antrostomy and anterior ethmoidectomy and a follow-up was scheduled to address sequelae of the destructive sinopulmonary lesions. This case report highlights a unique presentation of GPA with an insidious development of autoimmune sinonasal destruction in an adolescent female. This presentation is rare and highlights the importance of considering autoimmune disease in cases of tissue destruction where the etiology is not apparent, even in patients at low risk for autoimmune conditions.