LETHAL MALIGNANT midline granuloma is a relentless, destructive disorder of the facial tissues and other structures. It usually takes a progressive downhill course, lasting one to three years after the onset.1 Less than 150 cases of this rare disease of unknown etiology have been reported since McBride's first description2 in 1897. A close relationship has been postulated between lethal midline granuloma and Wegener's granulomatosis,3 which are both considered to be variants of periarteritis nodosa or other collagen-vascular diseases. A substantial number of patients with midline granuloma experience involvement of the lungs and other distant sites. Such involvements culminate in a terminal clinical state similar to that produced by progressive Wegener's granulomatosis.
Therapy of midline granuloma has been unsatisfactory despite transient response to steroids and temporary healing of individual lesions after x-radiation therapy.4 Long-term regression of progressive midline lethal granuloma by any systemic therapy has not yet
Greenspan EM. Cyclophosphamide-Prednisone Therapy of Lethal Midline Granuloma. JAMA. 1965;193(1):74-76. doi:10.1001/jama.1965.03090010080031