A localized sinusoidal lesion of granulomatosis with polyangiitis with extranasal involvement in a Syrian female: a rare case report

Abstract

Introduction: Granulomatosis with polyangiitis (GPA) is a rare, immunologically mediated systemic vasculitis that presents as an aseptic necrotizing granulomatous inflammation of the small and medium vessels. Case presentation: The authors report the case of a 47-year-old Syrian female smoker who was admitted to the hospital due to painless palpable masses in her left cheek and left upper lip. Her medical and family histories were unremarkable. Physical examination demonstrated facial asymmetry and bulging in the left cheek and left suborbital region, with marked limitation of the mouth opening and notable oozing from the maxillary sinus seen within the area of the extracted second premolar, in addition to swelling in the parotid gland region, which caused facial nerve weakness. Lab findings were significant for an elevated neutrophil count (16 400/mm3) and positivity of Cytoplasmic- Antineutrophil Cytoplasmic Autoantibody (c-ANCA). Microscopic examination revealed noncaseating necrotizing granulomas surrounded by histocytes and multinucleated giant cells. The disease kept its local invasion going despite the treatment with cyclophosphamide. Thus, surgical debridement was considered a notable improvement. Discussion: GPA is a systemic disease that usually affects multiple organs, especially the kidneys and the upper and lower respiratory tracts. The diagnosis of GPA is made based on a biopsy and the presence of c-ANCA. The treatment of GPA is patient-adjusted, and it usually consists of two major phases: induction and maintenance. However, surgery is preferred for patients who do not respond to pharmacotherapy. Conclusion: This article demonstrates a rare case of GPA in the head and neck region; that highlights the significant role of c-ANCA and histological exam in confirming the diagnosis as well as the importance of surgery when the disease is intractable.