Wegener granulomatosis with saddle nose deformity

For 10 weeks, a 34-year-old woman had refractory sinusitis, epistaxis, painful sores in her mouth with erosions in the nasal cartilage and palate, and otitis media in the left ear. She was subsequently found to have Wegener granulomatosis after punch biopsy of the lesions showed necrotizing vasculitis that involved small vessels. The patient was treated with hemodialysis, intravenous prednisone, and cyclophosphamide. Although her oral lesions had begun to resolve, saddle-nose deformity developed.

Oral manifestations in Wegener granulomatosis are rare.¹ The nose and paranasal sinuses are most commonly involved and are noted in about 64% to 80% of cases.¹ Saddle nose deformity develops in 10% to 25% of patients with nasal involvement.^1,2 Surgical reconstruction of the nose is complex and prone to complications and unsatisfactory results. However, surgical success rates are higher in patients in remission.

References:

REFERENCES:

1.

Gubbels SP, Barkhuizen A, Hwang PH. Head and neck manifestations of Wegener's granulomatosis.

Otolaryngol Clin N Am

. 2003;36:685-705.

2.

Chauhan S, D'Cruz S. Images in clinical medicine. Saddle nose deformity.

N Engl J Med

. 2007;356:2720.

Acknowledgment:

Michael E. Schott, Media Specialist, Mercy Medical Center.