An Unusual Case of Steroid Resistant IgG4-RD Affecting the Maxillary Alveolar Process

Abstract

Introduction Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune fibroinflammatory condition that is becoming increasingly recognized in the head and neck. I present a rare case of IgG4-RD originating in the alveolar process of the maxilla with orbital and maxillary sinus involvement that was unresponsive to traditional management. Case Presentation A 37-year-old Somalian woman presented with a 4-month history of progressive swelling of her right cheek and trismus. She had paresthesia and neuropathic pain affecting the V2 and V3 regions of the right trigeminal nerve. Testing of cranial nerve V revealed that she had decreased light-touch sensation over the site of swelling. Her hearing was reduced on the right side based on a whisper test. Investigations Ultrasound, magnetic resonance imaging, and computed tomography confirmed a mass centered on the right maxillary alveolus extending into the right buccal and masticator space, maxillary sinus, and orbit with involvement of the right infraorbital and greater palatine nerves. Histopathologic analysis revealed a storiform pattern of stromal fibrosis with a lymphoplasmacytic infiltrate and obliterative vasculitis. There was an increased number of IgG4+ plasma cells on immunostaining. Treatment She was started on corticosteroid but showed no response. Treatment with cyclophosphamide and rituximab provided improvement clinically and symptomatically. Discussion Clinically, IgG4-RD presents as a tumefactive, tissue-destructive lesion and often mimics malignant tumors, which can complicate diagnosis. IgG4-RD can be managed medically, and it is important to differentiate this disease from other pathologies in order to avoid invasive surgical management.