# Eosinophilic angiocentric fibrosis: A case report and a review of the literature

## TL;DR

This paper reports a rare case of Eosinophilic Angiocentric Fibrosis (EAF) and reviews the literature to highlight diagnostic features and management challenges.

## Contribution

The paper contributes a detailed case report of EAF involving multiple anatomical regions and emphasizes the importance of histopathological diagnosis.

## Key findings

- EAF is diagnosed by the characteristic 'onion-skin' pattern of perivascular eosinophil-rich inflammation and fibrosis.
- Medical and surgical interventions are used for EAF, but no standardized treatment guidelines exist.
- A thorough diagnostic approach, including imaging and histopathology, is essential due to the condition's varied presentation.

## Abstract

Eosinophilic Angiocentric Fibrosis (EAF) is a rare inflammatory condition affecting the upper respiratory and sinonasal tracts. This study aims to elaborate on the clinical and pathological features of a patient with extensive, rare EAF involving the sinonasal tract, subglottic region, and orbit.

A fifty-five-year-old female patient with a known case of bronchial asthma, hypothyroidism, and IgG-4-related necrotizing blepharitis presented to our clinic with a 14-year history of bilateral nasal obstruction and post-nasal drip despite medical and surgical intervention. Rhinoscopy revealed fibrosis and adhesion of the nasal cavity and nasopharynx, along with grade I subglottic stenosis. Functional endoscopic sinus surgery was performed to relieve her symptoms and obtain a specimen confirming the diagnosis of EAF.

Given the wide variety of differential diagnoses for bilateral nasal obstruction, imaging and histopathological evaluation are necessary to diagnose EAF. The characteristic onion-skin pattern reflecting the perivascular eosinophil-rich inflammatory infiltrate with subsequent concentric fibrosis is the gold standard in diagnosing these patients.

Despite advancements in clinical practice, a clear management guideline for patients with EAF has not yet been established. In our paper, we pool our management approach with the literature.

•Eosinophilic Angiocentric Fibrosis (EAF) is a rare inflammatory condition affecting the upper respiratory tract and paranasal sinuses.•EAF has a pathognomic onion-skin pattern, reflecting the perivascular eosinophils-rich inflammation with concentric fibrosis.•Symptoms of EAF include nasal obstruction, facial pain, post-nasal drip, and epistaxis; a thorough diagnostic approach is essential.•EAF can be managed medically or surgically; however, no clear guidelines exist, leading to a personalized patient-oriented decisions.

Eosinophilic Angiocentric Fibrosis (EAF) is a rare inflammatory condition affecting the upper respiratory tract and paranasal sinuses.

EAF has a pathognomic onion-skin pattern, reflecting the perivascular eosinophils-rich inflammation with concentric fibrosis.

Symptoms of EAF include nasal obstruction, facial pain, post-nasal drip, and epistaxis; a thorough diagnostic approach is essential.

EAF can be managed medically or surgically; however, no clear guidelines exist, leading to a personalized patient-oriented decisions.