RESUMO
INTRODUCTION: Centrofacial granulomas have several etiologies, which poses diagnostic difficulties and delays in management. Herein, we report a confusing case revealing granulomatosis with polyangiitis (GPA) in its localized form. CASE PRESENTATION: A 76-year-old man presented with a pruritic centrofacial placard that had been evolving for 3 years. On examination, there was a centrofacial infiltrated and erythematous papulonodular placard. Skin biopsies were not conclusive. The immunological assessment was negative. The evolution was marked by the extension of the placard, the destruction of the nasal pyramid and nasal mutilation. Only surgical biopsy revealed dermohypodermal, cartilaginous and endonasal non-necrotizing granulomatous tuberculoid inflammation with leukocytoclastic and necrotizing vascularitis. The diagnosis of GPA in its localized form was retained. Treatment with prednisone was initiated combined with monthly boluses of cyclophosphamide. The assessment for systemic involvement remained negative during the follow-up. CLINICAL DISCUSSION: Localized forms represent up to 29% of GPA cases. There are clinical, but also biological differences, since ANCA are found in more than 90% of diffuse forms and only in 50-78% of localized forms. Our case may represent a rare distinctive subset of GPA limited to the facial region and upper airway mucosa but showing a locally aggressive behaviour leading to cartilage and bony destruction. CONCLUSION: It is necessary to evoke GPA in its localized form and to perform multiple deep biopsies in front of any facial granulomatosis. Early diagnosis and appropriate treatment prevent mutilating and disfiguring sequelae.
