BACKGROUND Nasal deformity is a common disorder in Wegener granulomatosis, which is produced by polyangitis of medium-sized vessels. This process may cause necrosis and destruction of the nasal mucosa and osteocartilaginous framework. At this time, there is a lack of published data on nasal deformities associated with Wegener granulomatosis. METHODS We present a 53-year-old woman with complet...

Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no repor...

We report the case of a 57-year-old man with a known history of antineutrophil cytoplasmic antibody-positive Wegener granulomatosis with initial involvement of the upper and lower respiratory tract. MR imaging of the brain was performed because of new onset CNS symptoms (nausea, altered mental status). The images revealed a mass in the fourth ventricle. The mass diminished in size after systemi...

Transalar encephaloceles are rare lesions that do not fit the standard classification of basal encephaloceles. Typically, these lesions present in adulthood, with nonspecific symptoms. We report here a case of a patient with Wegener disease in whom a large transalar encephalocele posterior to the sinus was noted when he was preoperative for left maxillary sinus surgery. The encephalocele demons...

We report on a patient with biopsy proven systemic Wegener's granulomatosis (WG) with a granulomatous necrotising manifestation of WG in the liver, lung, parotid gland and skin with subsequent death of liver failure. Liver involvement in WG is an exceedingly rare, though potentially fatal, organ manifestation of WG.