The woman with a swollen tongue : A rare case of an ANCA-negative localized eosinophil-rich granulomatous inflammation affecting solely the tongue.

Eosinophilic granulomatosis with polyangiitis (EGPA, previously known as Churg-Strauss syndrome) is an antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often presenting with chronic rhinosinusitis, pulmonary infiltrates, gastrointestinal and cardiac disorders, or renal lesions. Sinonasal and inner ear manifestations are common, but other affections of the head and neck area are rarely reported. Here we report a case of a young woman with a histopathological diagnosis of eosinophil-rich granulomatous inflammation affecting solely the tongue without other local or systemic lesions. It suggests histopathologically a localized EGPA according to the 1992 Chapel Hill classification but does not formally meet the recent 2022 joint classification criteria for EGPA of the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR). In this report, we also describe the difficulty associated with the treatment.