A rare manifestation of Wegener´s disease: scleromalacia perforans

A 45-year-old woman with no known medical history presented to our hospital for three weeks of reduced vision, painful eye, and rhinorrhoea. Slit lamp examination of the right eye showed temporal scleral thinning with visible uveal tissue, inferior corneal ulcer, superior corneal infiltrates, normal deepness of the anterior chamber and advanced cataract. Slit lamp examination of the left eye was unmarkable. Routine laboratory tests revealed high c-reactive protein (CRP) (90 mg/l), a high sedimentation rate (50 mm/hr), and a positive antineutrophil cytoplasmic antibody value. Nasal mucosal biopsy revealed granulomatous vasculitis. The patient was treated with prednisolone and rituximab. Azathioprine was given for maintenance. The patient died of respiratory complications.