악하부 농양으로 발병한 육아종증 다발 혈관염 1예

• 영문명: A Case of Granulomatosis with Polyangiitis with Submandibular Abscess as Primary Manifestation
• 발행기관: 대한두경부종양학회
• 저자명: 전현웅 홍성만 정진혁 지용배
• 간행물 정보: 『대한두경부종양학회지』제39권 제1호, 27~31쪽, 전체 5쪽
• 주제분류: 의약학 > 종양학
• 파일형태: PDF
• 발행일자: 2023.05.31

영문 초록

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare immunological small vessel disease which usually affects respiratory tract and kidneys. However, salivary gland involvement in GPA is rare as a primary manifestation. We report a case of GPA with the primary presentation of submandibular gland involvement. A 48-year-old female patient presented submandibular swelling with a skin defect that lasted for 1 month. Although the biopsy result was chronic inflammation, the skin defect did not heal for a month. Further imaging study revealed multiple lung and renal masses. More clinical manifestations such as gingivitis, ischemic change of finger joint and nasal tip skin, and positive c-ANCA test was presented. Additional biopsy was made at the submandibular gland, lung, and finger skin. The patient was finally diagnosed with GPA and treated with steroid pulse therapy and cyclophosphamide. The patient showed improvement of prior clinical symptoms.

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키워드

Granulomatosis with polyangiitis Submandibular gland