[Atypical purpuric oedema of the nose during granulomatosis with polyangiitis]. / Un œdème purpurique du nez atypique au cours d'une granulomatose avec polyangéite.

ABSTRACT

INTRODUCTION: Granulomatosis with polyangeitis or Wegener's disease is a necrotizing vasculitis of small and medium vessels associated with antineutrophil cytoplasmic autoantibodies (ANCA). The most frequent sites are lung, ear, nose and throat and kidney. PATIENTS AND METHODS: We report the case of a 47-year-old woman presenting purpuric oedematous plaque with bullous detachment of the nose and hospitalised for the assessment of two suspicious neoplastic lung lesions discovered as a result of a recent stroke and repeated seromucosal otitis. Granulomatosis with polyangeitis was suspected because of multiple systemic lesions. The histopathology of skin lesions and laboratory investigation results were consistent with this diagnosis. A favourable outcome was achieved with corticosteroids and rituximab. DISCUSSION: The diagnosis of GPA is based on criteria established by the American College of Rheumatology. The cutaneous clinical aspect described in our case confirms the polymorphism of the cutaneous lesions possibly associated with this disease. They are rarely isolated but, in some cases, allow early diagnosis with improved prognosis, which remains severe in the absence of treatment.