Eosinophilic angiocentric fibrosis of the upper respiratory tract: a mucosal variant of granuloma faciale? A report of three cases

Abstract

Three patients with an unusual stenosing lesion affecting the upper respiratory tract are described. The lesion involved the mucosa of the nasal septum and lateral nasal wall in two cases and the mucosa of the sub-glottic region of the larynx in one. The lesion leads to thickening of the sub-mucosal connective tissues by an usual pathological process. It begins as a focal peri-vascular sub-epithelial exudate of eosinophils accompanied by collections of plasma cells and lymphocytes without fibrinoid necrosis. These foci progress to form extensive areas of peri-vascular fibrosis showing a characteristic angiocentric whorling with an 'onion-skin' pattern. This process leads to thickening and matting of the mucosa which becomes adherent to the underlying structures. As fibrosis progresses, the lympho-plasmacytic component regresses but the eosinophils remain. The fibrosis does not resolve and the consequent stenosis requires surgical relief. No other site appears to be affected by this process, but one case was associated with granuloma faciale. The cause is unknown. We have given the term 'eosinophilic angiocentric fibrosis of the upper respiratory tract' to this condition to encompass its cardinal features.