Treatment of "idiopathic midline destructive disease" by irradiation. A case report.

An unusual case with an aggressive destructive granulomatous lesion of the maxilla is reported. Although the possibility of infection and neoplasm could be ruled out, a definite diagnosis could not be established even by repeated biopsies which showed the lesion to be a non-specific inflammatory process. Since Wegener's granulomatosis was most unlikely, the patient was treated by radiotherapy which caused rapid remission of the lesion with no sign of recurrence after 4 years. The clinical and histological findings as well as the responsiveness to the treatment were most indicative of idiopathic midline destructive disease. Effective management of lethal midline granuloma of unknown aetiology is discussed.