A 46 yo female PMH of RA, pemphigus vulgaris on chronic steroids, persistent rhinorrhea, and remote cocaine use was transferred from an outside hospital with 20 days of progressively worsening flu-like symptoms, nasal discharge and watery eyes with facial swelling and pain. \xa0Her symptoms had evolved over the previous week to include worsening, muffled hearing in Lt>Rt ear, and yellow/green drainage from a Rt infraorbital ulcer. Patient was started 2 weeks prior on an empiric course of antibiotics without improvement in symptoms. Physical exam revealed a mildly ill appearing woman with normal vital signs and a saddle nose deformity (first noted by the patient 1 year ago). Patient had swelling under both eyes Rt>Lt with a fistulous tract 1 cm below the Rt inferior lid margin. There was minimal surrounding erythema, fluctuance, and significant tenderness. Purulent fluid was readily expressed. Lt tympanic membrane was extremely injected and thickened with Weber lateralization to the left and air greater than bone conduction was noted bilaterally. Labs were significant for ANA +<1:40, anti-DNA negative, ANCA negative, MPO and PR3 were mildly elevated at 117 and 144 (both normal ranges of 0-99). UA and BUN/Cr were unremarkable. Sinus biopsy demonstrated marked ulceration, granulation tissues and acute and chronic inflammation without evidence of vasculitis. Patient was discharged but returned before follow up appointment due to worsening symptoms. On exam patient had a small RLE hemorrhagic, crusted plaque. Plaque biopsy demonstrated suppurative granulomatous inflammation with background vasculitis. Patient was started on GPA dosing steroids and cyclophosphamide/mesna. She was discharged home and at follow-up 3 weeks later she reported increased energy, improved hearing and decreased pain and swelling around her eyes. She continued follow up with ENT and rheumatology and continues on monthly cyclophosphamide infusions.
DeFelice, Nathaniel; Sara Marian S. Lucking; and Peggy Beeley. "Clinical vignette: A case of peculiar midline necrotizing rhinosinusitis." (2014). https://digitalrepository.unm.edu/hostpitalmed_pubs/21