Vasculitis Presenting as an Infratemporal Fossa Mass

Background: Vasculitides are rare group of autoimmune diseases that commonly involve the larynx, ears and sinonasal mucosa. We present an unusual case of an infratemporal mass that was eventually determined to be the result of ANCA-negative small vessel vasculitis.

Case Presentation: A 51-year-old male presented to the emergency department with 2 to 3 months of right cheek pain and facial swelling. He was discharged with a 10-day Augmentin course, daily Flonase and was scheduled for otolaryngology outpatient follow-up. The patient presented back to the emergency department nine days later and examination showed diminished sensation about the right V2 distribution. A MRI brain was obtained revealing a mass within the right infratemporal and pterygopalatine fossae extending into the inferior right orbital fissure along V2 and the vidian nerve concerning for malignancy. The patient was taken to the operating room for biopsy. Intraoperatively he had a necrotic appearing internal maxillary artery. Histology demonstrated multiple arteries with partial to complete luminal obliteration with associated loosely formed non-necrotizing granulomas, consistent with a granulomatous vasculitis. Serum studies demonstrated a positive antinuclear antibody, but negative ANCA. He was evaluated by infectious diseases, and workup of HIV, hepatitis B and C, tuberculosis, and diverse opportunistic organisms were all negative. The patient was referred to rheumatology and nephrology and was diagnosed with ANCA-negative small vessel vasculitis and started immunosuppressive treatment with prednisone and mycophenolate.

Discussion/Conclusion: Vasculitides can affect small, medium, and large arteries of the head and neck, and there are limited case reports of vasculitis presenting as an infratemporal mass. Despite its rarity, upon diagnosis, referral to nephrology and/or rheumatology is recommended to rule out systemic involvement of the disease and to prevent treatment delays which may lead to destruction of vital organs.

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Artikel online veröffentlicht:
01. Februar 2023