Roberts P F, McCann B G
Histopathology. 1985 Nov;9(11):1217-25. doi: 10.1111/j.1365-2559.1985.tb02801.x.
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.
本文描述了3例患有影响上呼吸道的罕见狭窄性病变的患者。2例病变累及鼻中隔和鼻外侧壁黏膜,1例累及喉声门下区黏膜。该病变通过一种特殊的病理过程导致黏膜下结缔组织增厚。病变起始为局灶性血管周围上皮下嗜酸性粒细胞渗出,伴有浆细胞和淋巴细胞聚集,无纤维蛋白样坏死。这些病灶进展形成广泛的血管周围纤维化区域,呈现特征性的以血管为中心的漩涡状“洋葱皮”样图案。这一过程导致黏膜增厚并相互交织,进而与下方结构粘连。随着纤维化进展,淋巴细胞和浆细胞成分减少,但嗜酸性粒细胞仍存在。纤维化不会消退,由此导致的狭窄需要手术缓解。该过程似乎未累及其他部位,但1例与面部肉芽肿有关。病因不明。我们将这种疾病命名为“上呼吸道嗜酸性血管中心性纤维化”,以涵盖其主要特征。