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Three patients seen at the Medicine Department of Del Salvador Hospital between 1986 and 1990 are reported. All had a history of purulent or bloody nasal discharge and recurrent sinusitis, before the appearance of progressive and painful destructive phenomena that affected the rhino faucial region. The diagnosis was made with the histopathological study that disclosed and angiocentric polymorphic infiltrates and perivascular necrosis. The three patients received similar treatment consistent in prednisone 1 mg/kg/day per os, cyclophosphamide 2 mg/kg/day per os and local radiotherapy. The response to therapy was bad and survival was less than three months. It is concluded that although this entity is infrequent, its severity requires and aggressive workup and management by a multidisciplinary team. Notwithstanding the mortality remains to be high.
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