Background: The aim of this study was to assess the principal signs and symptoms for severe necrotizing midline nasal lesions and give diagnostic and management algorithms. Methods: Literature review and retrospective chart review were performed. Three patients with Wegener's granulomatosis (WG), six patients with sarcoid, eight patients with cocaine abuse, and one lymphoma patient were analyzed with respect to symptom complexes, laboratory results, and radiographic findings. Based on computed tomography findings, a nasal destruction score was tabulated for each patient. Results: All diseases essentially had similar signs and symptoms within the head and neck but several extranasal sites were suggestive of specific etiologies. Serological laboratory testing was diagnostic in only two of the six sarcoid patients and two of the three WG patients. There were no specific tests associated with lymphoma or cocaine abuse, although erythrocyte sedimentation rate was consistently and significantly elevated in the latter group. Biopsy confirmed disease was found in one of the three sarcoid patients, in one of the three WG patients, and in one out of one of the lymphoma patients. Nasal destruction scores were highest in WG and lymphoma patients, intermediate in cocaine abuse patients, and lowest in patients with sarcoidosis. Two of the three WG patients had extensive neoosteogenesis. One out of one lymphoma and five of the eight cocaine abuse patients had oronasal or oroantral fistulas. Conclusion: Laboratory tests and biopsies were consistently unreliable in all diseases. Repeat studies should be performed in all cases of negative results if clinical suspicion is high. Neo-osteogenesis and mastoid disease were associated with WG when compared with patients with similar levels of nasal destruction due to other etiologies. Laryngeal and dermatologic changes without significant nasal bony abnormalities raised suspicion for sarcoidosis. Hard and soft palate defects were associated with cocaine abuse and extranodal nasal lymphoma.