Sinonasal Eosinophilic Angiocentric Fibrosis: A Report of Four Cases and Review of Literature

Jain, Reena; Robblee, Jennifer; O’Sullivan‐Mejia, Emerald; Lea, Jane; Heller, Andrew J.; Faquin, William C.; Powers, Celeste N.

doi:10.1007/s12105-008-0077-y

Cited by 38 publications

(39 citation statements)

References 24 publications

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“…Absence of geographic necrosis, necrotizing vasculitis and granulomatous inflammation excludes Wegener's granulomatosis (WG) and Churg-Strauss syndrome (CS). Also, EAF lacks systemic vasculitis which is a participant of CS and mucosa over the EAF lesion does not show necrosis which is usually seen in WG lesions (4,10,11). Blood test positivity for c-ANCA and p-ANCA supports the diagnosis of WG and CS, respectively (4,11).…”

Section: Discussionmentioning

confidence: 89%

“…The histology of EAF is pathognomonic and characterized by perivascular inflammatory cell infiltration with progressive fibrosis around small vessels leading to the typical "onion-skin" pattern (3,4). Eosinophils are the predominant cells with some degrees of plasma cells and lymphocytes of early inflammatory lesions.…”

Section: Discussionmentioning

confidence: 99%

“…Medical therapy is found to be ineffective but may cause symptomatic relief (2,8). Intranasal, intralesional or systemic corticosteroids, antihistamines, dapsone, hydroxychloroquine, azathiopurine and tamoxiphen are trial therapies in the literature but none of them were found to be as effective as surgery (2,4,5,8). Surgical excision of the lesion should be complete resection as far as possible (2,7).…”

Section: Discussionmentioning

confidence: 99%

“…EAF was first described by Holmes and Panje in 1983 as "intranasal granulome faciale" and in 1985 Roberts and McCann (2, 3) reported 3 similar intranasal cases with detailed histopathological behavior. Although EAF has a clear clinical and histological appearance, its pathogenesis is still controversial (2,4). EAF typically presents in young to middle-aged females with a submucosal inflammatory, fibrosing tumorlike lesion causing slowly progressive upper airway symptoms.…”

Section: Introductionmentioning

confidence: 99%

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Rare Pathology of Sinonasal Tract; Eosinophilic Angiocentric Fibrosis

Kayahan¹,

Cabbarzade²,

Özer³

et al. 2014

Turk Arch Otolaryngol

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Eosinophilic angiocentric fibrosis (EAF) is a relatively rare, benign and slowly progressive fibroinflammatory disorder, which mainly involves the upper respiratory tract. EAF was first described by Holmes and Panje in 1983. EAF is typically seen in young to middle-aged females and it has a slowly progressive nature. As well as the sinonasal region the nasal septum is mainly the most common localization of EAF, involvement of the larynx and orbita was also described in literature. Symptoms vary with the involved area but nasal obstruction, epistaxis and epiphora are the most common complaints. The etiology of EAF is not clear and it is diagnosed by specific histopathological features. Histologically, the lesion is characterized by a perivascular, eosinophil-rich inflammatory infiltrate and progressive fibrosis which appears as a whirling "onion-skin" pattern. In this paper; a case of sinonasal EAF is reported with the clinical course, radiological and histological features, etiology and treatment of these lesions based on updated literature. Key Words: Eosinophilic angiocentric, fibrosis, sinonasal AbstractThis study was presented as a poster at the

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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Rare Pathology of Sinonasal Tract; Eosinophilic Angiocentric Fibrosis

Kayahan¹,

Cabbarzade²,

Özer³

et al. 2014

Turk Arch Otolaryngol

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“…Other than the nose and sinuses, the other structures such as the eye orbit, gums, larynx, and upper trachea can be involved as well. [2][3][4][5] However, lower airway involvement is very rare, and was reported for the first time in 2011 by Deshpande et al 6 The precise etiology of EAF still remains unclear. Atopy, immunologic disease, previous surgery, and trauma have been suggested to be predisposing factors, but the evidence for any of these origins is weak.…”

Section: Introductionmentioning

confidence: 99%

Unexplained Persistent Dyspnea in a Young Woman With Eosinophilic Angiocentric Fibrosis

Kim¹,

Kim²,

Kim³

et al. 2013

Respir Care

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Eosinophilic angiocentric fibrosis (EAF) is a rare inflammatory disease that primarily involves the nose and sinuses. Involvement of the eye orbit and larynx has also been described. However, it is very rare for this disease to involve the lower respiratory tract and cause dyspnea. We describe a rare case of EAF involving the lower respiratory tract with airway narrowing. A 29-year-old female with a 7-year history of nasal obstruction presented with unexplained persistent dyspnea. EAF was diagnosed via endoscopic biopsy of an irregular mucosal lesion in the posterior wall of the right maxillary sinus. Chest computed tomography and bronchoscopy showed a diffuse inflammatory narrowing of the airway in the tracheobronchial trees. EAF can affect lower respiratory tracts with airway narrowing that can be characterized by dyspnea. We must consider narrowing of the lower respiratory tracts in patients with EAF complaining of unexplained persistent dyspnea.

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Cutaneous Vasculitis

Levell

Mukhtyar

2016

Rook's Textbook of Dermatology, Ninth Edition

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Overview Vasculitis is usually a multisystem disorder that presents in a myriad of ways. Diagnosis is based on a detailed history and careful examination. Patients may present to different specialties and their care should be led by a multidisciplinary team involving physicians with a specialist interest in vasculitis. Vasculitis can be classified by aetiology or by calibre of the vessel involved. The accepted nomenclature was defined by an international consensus meeting held in 2012; cutaneous vasculitides are considered under the umbrella of ‘single organ vasculitis’, although the classification has been expanded in this chapter. Ill‐defined entities that do not have an identified pathological basis have not been included. It should be recognized that names and classifications will change in the future with greater understanding of the underlying mechanisms of disease. The treatment of primary vasculitis involves immunosuppression. The balance between disease severity and adverse effects of treatment requires expertise and experience in management of these rare conditions. Secondary vasculitis may be due to infection, drugs, malignancy or inflammatory disease. Treating the underlying condition may resolve the vasculitis.

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Sinonasal Eosinophilic Angiocentric Fibrosis: A Report of Four Cases and Review of Literature

Abstract: Eosinophilic angiocentric fibrosis (EAF) is a rare, benign condition of unknown etiology involving the sinonasal tract and the upper respiratory airways, and rarely, larynx, and orbit. We report four cases of EAF identified, in three women and one man, aged 31, 57,

Cited by 38 publications

References 24 publications

Rare Pathology of Sinonasal Tract; Eosinophilic Angiocentric Fibrosis

Rare Pathology of Sinonasal Tract; Eosinophilic Angiocentric Fibrosis

Unexplained Persistent Dyspnea in a Young Woman With Eosinophilic Angiocentric Fibrosis

Cutaneous Vasculitis

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