Wegener's Granulomatosis with Eosinophilia

Wegener's granulomatosis with eosinophilia both in blood and tissue samples has been rarely reported. A 25-year-old woman developed fever, granulomatous inflammation of nasal mucosa, pulmonary hemorrhage, suprascleritis, and cutaneous vasculitis. She showed eosinophilia in blood and biospy samples of skin, nasal mucosa, and lung. She was positive for proteinase 3-antineutrophil cytoplasmic antibody. She had no history of asthma. We diagnosed her as Wegener's granulomatosis with eosinophilia rather than Churg-Strauss syndrome. Her mucocutaneous and pulmonary lesions were successfully treated with high-dose corticosteroid and methotrexate, but her suprascleritis required pulsed cyclophosphamide.