Sunde Jumin, Alexander Kathrina A, Reddy Vishnu V B, Woodworth Bradford A
The University of Alabama School of Medicine, University of Alabama at Birmingham, BDB 563, Birmingham, AL 35294, USA.
Head Neck Pathol. 2010 Sep;4(3):246-8. doi: 10.1007/s12105-010-0185-3. Epub 2010 Jun 9.
Eosinophilc angiocentric fibrosis is a rare fibrosing vasculitis of unknown etiology that is progressive and potentially disfiguring. It has a predilection for the upper airways presenting most commonly as an obstructing mass lesion that is diagnosed histologically. Thus far, it has been nonfatal in the more than 40 reported cases; however, subglottic and ocular involvement has resulted in significant morbidity in several patients. Treatment has been challenging with persistent disease in most cases. The current case is a prototypical presentation with a limited nasal septal lesion providing the opportunity to discuss clinically relevant issues and increase awareness.
嗜酸性粒细胞性血管中心性纤维化是一种病因不明的罕见纤维化性血管炎,呈进行性发展,可能导致容貌毁损。它好发于上呼吸道,最常见的表现是阻塞性肿块病变,需通过组织学诊断。迄今为止,在已报道的40多例病例中均未出现致命情况;然而,声门下和眼部受累已导致数名患者出现严重并发症。在大多数情况下,治疗具有挑战性,疾病持续存在。本病例是一个典型表现,鼻中隔病变局限,为讨论临床相关问题和提高认识提供了机会。