A Case of Granulomatosis with Polyangiitis with Submandibular Abscess as Primary Manifestation

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.