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CASE REPORT
Year : 2019  |  Volume : 12  |  Issue : 1  |  Page : 69-71  

Eosinophilic angiocentric fibrosis: A case report with review of literature


1 Department of Pathology, BVDU Medical College and Hospital, Sangli, Maharashtra, India
2 Department of Transfusion Medicine, Government Medical College, Miraj, Maharashtra, India

Date of Submission11-Feb-2018
Date of Acceptance02-Apr-2018
Date of Web Publication22-Jan-2019

Correspondence Address:
Amruta Ashok Patil
Department of Pathology, BVDU Medical College and Hospital, Sangli, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/mjdrdypu.mjdrdypu_30_18

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  Abstract 


Eosinophilic angiocentric fibrosis (EAF) is a rare benign condition mainly affecting the nasal cavity. It has characteristic histopathological features as eosinophil-rich perivascular onion skin-like fibrosis. Ours was an interesting case of the right nasal cavity mass in middle-aged male who presented with nasal obstruction. Histopathological features were characteristic of EAF. Treatment with excision of lesion and corticosteroids had no recurrence on 6-month follow-up.

Keywords: Eosinophilic angiocentric fibrosis, nasal septum, Tumor like lesion


How to cite this article:
Patil AA, More SJ. Eosinophilic angiocentric fibrosis: A case report with review of literature. Med J DY Patil Vidyapeeth 2019;12:69-71

How to cite this URL:
Patil AA, More SJ. Eosinophilic angiocentric fibrosis: A case report with review of literature. Med J DY Patil Vidyapeeth [serial online] 2019 [cited 2019 Feb 15];12:69-71. Available from: http://www.mjdrdypv.org/text.asp?2019/12/1/69/250438




  Introduction Top


Eosinophilic angiocentric fibrosis (EAF) is an uncommon benign condition first described by Holmes and Panje in 1983 under the name intranasal granuloma faciale (GF).[1] The nomenclature EAF was first used by Roberts and McCann in 1986.[2] Clinically, EAF presents with slowly progressive airway obstructive symptoms in middle-aged patients. Histologically, it shows dense fibrotic stroma with perivascular onion skin-like fibrosis and dense infiltration by eosinophils, lymphocytes, and plasma cells. Necrosis and foreign body-type giant cells are not seen. Medical treatment can be helpful in initial lesions, but surgical intervention is needed to resolve the obstructive symptoms. We are here describing the case of primary nasal septum tumor-like lesion in an elderly male, diagnosed as EAF based on histological findings with discussion on literature of EAF.


  Case Report Top


A 60 years male presented with progressive nasal obstruction for 3 months. On per speculum examination, a mass was seen arising at nasal septum in the right nasal cavity obstructing the air flow. Computed tomography scan revealed soft-tissue opacification at the septum having ill-defined margins with cartilaginous and superficial bony erosion. Complete blood counts, blood biochemistry, erythrocyte sedimentation rate, and coagulation parameters were in normal ranges. Excision of the mass was done by endoscopic resection.

We received a well-circumscribed, firm, grayish-tan mass measuring 3.5 cm × 2.5 cm × 2.0 cm with entrapped nasal cartilage in the center of the mass [Figure 1]. Hematoxylin and eosin-stained sections showed fibroinflammatory lesion with dense fibrotic stroma [Figure 2]. There was perivascular onion skin-like fibrosis and infiltration mainly by eosinophils, lymphocytes, and plasma cells [Figure 3] and [Figure 4]. The lesion is seen to involve adjacent skeletal muscle [[Figure 4], inset]. Fibrinoid necrosis, vasculitis, or true granuloma formation were absent in all the sections.
Figure 1: Well-circumscribed, firm, grayish-tan mass with entrapped nasal cartilage in the center of the mass

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Figure 2: Fibroinflammatory lesion showing onion skin fibrosis (H and E, ×20)

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Figure 3: Perivascular onion skin fibrosis with dense infiltration by lymphocytes, plasma cells, and eosinophils in inset (H and E, ×40)

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Figure 4: Entrapped septal cartilage and adjacent skeletal muscle (Inset) (H and E, ×40)

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After the diagnosis of EAF was established, the patient was started on corticosteroids. On 6-month follow-up, he had no recurrence.


  Discussion Top


EAF is a rare benign disorder with unknown etiology, mainly affecting upper respiratory and sinonasal tract including the nasal cavity. EAF generally presents as submucosal inflammatory and fibrosing tumor-like lesion. Roberts and McCann in their case report of three patients and in a postscript of two additional patients in 1985 and 1997 described this entity.[1],[2] Nasal cavity is a common site, but it can affect other regions in head and neck such as orbit, subglottis, and trachea, leading to dysphonia or airway compression.[2],[3] Till date, >51 patients are diagnosed with EAF in medical literature, and the nasal septum was the most commonly affected location. No apparent sex predilection was observed (male:female = 2:3).[4]

The etiology of EAF is unknown though some authors have thought of autoimmune or allergic or trauma as a cause. Some authors are also working on theory that EAF is a part of spectrum of IgG4-related disease.[2] Patients generally present with symptoms of nasal obstruction and stuffiness, epistaxis, breathing difficulties, epiphora, and proptosis; however, these symptoms tend to be chronic and progressive. Radiographic evaluations are also usually nonspecific, showing clouding and opacification of the nasal cavity and sinuses with or without bony erosion, sclerosis, and focal destruction of the surrounding bone.[2]

The histopathological findings depend on the stage of the disease, but there is no clear boundary between early and late lesions. Early disease shows eosinophil-rich perivascular fibrosing inflammatory lesion, while late lesions show dense perivascular “onion-skin” fibrosis with decrease in inflammatory cells. It is likely to see both early and later stages simultaneously at a single biopsy.[4]

The diagnosis will depend on the presence of classic histological findings of EAF, but if features such as fibrinoid necrosis and granuloma formation are seen; then, differential diagnosis to be considered is Wegener's granulomatosis, Churg–Strauss syndrome, Kimura disease, GF, juvenile angiofibroma, and IgG4-related disease.

Wegener's granulomatosis shows granulomatous vasculitis and geographic necrosis with positivity for c-ANCA. In Chrug–Strauss syndrome, there is eosinophilic vasculitis, but it shows fibrinoid necrosis with granulomas. Kimura disease shows dense lymphoid aggregates with prominent germinal centers, though it shows fibrosis but lacks the typical angiocentric whorled pattern characteristic of EAF.[5]

GF is a benign cutaneous disease of unknown etiology characterized by sharply circumscribed plaques and skin nodules, with a predilection for the facial region, but mucosal involvement is exceedingly uncommon. Theory of EAF as an extracutaneous lesion of GF was also proposed by some authors.[4] Histologically, GF shows infiltration by eosinophils, neutrophils, and lymphoid cells sparing the surface. Early lesion of GF shows vasculitis. Although fibrosis is seen, it is neither prominent nor concentrically layered.[6] Our patient had no skin plaques or nodules. Furthermore, the characteristic microscopic findings ruled out the diagnosis of GF.

Sinonasal tract EAF is an indolent and progressive disease. Surgery is more useful in patients presenting with obstructing symptoms. Although recurrence is rare, it usually develops at the primary lesion site; hence, it is thought to be a progressive disease. In some patients, the disease process may progress even with the use of corticosteroid therapy; hence, regular follow-up is essential.[7]


  Conclusion Top


EAF is a rare, indolent, progressive benign disorder which may cause local tissue destruction and have predilection for upper respiratory tract. The characteristic histological findings permit the diagnosis of EAF. It is important to treat the lesion by total resection and corticosteroid therapy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Holmes DK, Panje WR. Intranasal granuloma faciale. Am J Otolaryngol 1983;4:184-6.  Back to cited text no. 1
    
2.
Roberts PF, McCann BG. Eosinophilic angiocentric fibrosis of the upper respiratory tract: A mucosal variant of granuloma faciale? A report of three cases. Histopathology 1985;9:1217-25.  Back to cited text no. 2
    
3.
Fageeh NA, Mai KT, Odell PF. Eosinophilic angiocentric fibrosis of the subglottic region of the larynx and upper trachea. J Otolaryngol 1996;25:276-8.  Back to cited text no. 3
    
4.
Li Y, Liu H, Han D, Zang H, Wang T, Hu B, et al. Eosinophilic angiocentric fibrosis of the nasal septum. Case Rep Otolaryngol 2013;2013:267285.   Back to cited text no. 4
    
5.
Pereira EM, Millas I, Reis-Filho JS, Maeda SA, Franco M. Eosinophilic angiocentric fibrosis of the sinonasal tract: Report on the clinicopathologic features of a case and review of the literature. Head Neck 2002;24:307-11.  Back to cited text no. 5
    
6.
Ackerman A. Vasculitis in Histologic Diagnosis of Inflammatory Skin Disease: A Method by Pattern Analysis. Philadelphia, PA: Lea & Febiger; 1978. p. 333-84.  Back to cited text no. 6
    
7.
Thompson LD, Heffner DK. Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases. Am J Clin Pathol 2001;115:243-8.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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