Idiopathic midline destructive disease (IMDD): a subgroup of patients with the "midline granuloma" syndrome.

"Midline granuloma syndrome" encompasses a number of specific clinicopathologic entities, such as "Wegener's granulomatosis, midline malignant reticulosis, lymphoma, nasal carcinoma and a broad spectrum of infectious diseases. In the course of studying a number of patients presenting with destructive lesions of the upper respiratory tract, 11 cases emerged as having unique clinicopathologic features. These are: 1) presence of locally destructive lesions which are always restricted to the upper respiratory tract. 2) Absence of systemic disease during a follow-up period of six months to 18 years (mean 7.3 years). On examination of repeated biopsy specimens the histopathologic picture consisted of acute and chronic inflammation with variable amounts of necrosis. Granulomata were seen in one case. Malignant or atypical cells were invariably absent. Frank vasculitis was not seen, although in five of the patients inflammatory cells in the wall of small vessels were conspicuous 4) Inability to demonstrate an infectious origin by culture or special stains. Radiation therapy has proven effective in patients with this entity, for which the term "Idiopathic Midline Destructive Disease" (IMDD) is proposed.