Indian Journal of Pathology and Microbiology

CASE REPORT
Year
: 2015  |  Volume : 58  |  Issue : 1  |  Page : 77--79

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


Santhi Radhakrishnan1, Namrata G Adulkar2, Viji Rangarajan3,  
1 Department of Ocular Pathology, Aravind Eye Hospital, Madurai, Tamil Nadu, India
2 Department of Orbit, Oculoplasty and Oncology, Aravind Eye Hospital, Madurai, Tamil Nadu, India
3 Department of Orbit and Oculoplasty, Aravind Eye Hospital, Coimbatore, Tamil Nadu, India

Correspondence Address:
Dr. Namrata G Adulkar
Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, 1, Anna Nagar, Madurai - 652 020, Tamil Nadu
India

Abstract

Eosinophilic angiocentric fibrosis is a rare condition presenting with tumefactive lesions of the nasal cavity, paranasal sinuses, upper respiratory tract and rarely the orbit; and has characteristic histopathological features. We report the case of a 38-year-old lady with a mass lesion in right orbit. Histopathological examination of the biopsy specimen revealed extensive areas of perivascular fibrosis showing a characteristic angiocentric whirling with onion skin pattern; along with perivascular exudates of eosinophils accompanied by collection of plasma cells and lymphocytes. Rheumatology review and autoimmune screening was negative. She underwent surgical debulking followed by tapering course of oral steroids and was completely free of orbital disease at her last follow-up visit.



How to cite this article:
Radhakrishnan S, Adulkar NG, Rangarajan V. Eosinophilic angiocentric fibrosis of the orbit: A case report and review of literature.Indian J Pathol Microbiol 2015;58:77-79


How to cite this URL:
Radhakrishnan S, Adulkar NG, Rangarajan V. Eosinophilic angiocentric fibrosis of the orbit: A case report and review of literature. Indian J Pathol Microbiol [serial online] 2015 [cited 2021 Apr 18 ];58:77-79
Available from: https://www.ijpmonline.org/text.asp?2015/58/1/77/151194


Full Text

 INTRODUCTION



Eosinophilic angiocentric fibrosis (EAF) is an uncommon clinical entity; characterized by tumefactive lesions of the nasal cavity, paranasal sinuses, upper respiratory tract and rarely the orbit. Until date, only six cases, primarily involving the orbit, have been described in world literature. We report a case of a 38 years lady with EAF in the right orbit and discuss the relevant literature. The case study was undertaken in compliance with the Declaration of Helsinki and was approved by the Hospital's Ethics Committee.

 Case Report



A 38-years old lady presented to us with right inferior orbital mass of 5 months duration. Her best-corrected visual acuity was 6/6, N6 in both eyes. On examination, she had firm nontender mass lesion palpable in the inferolateral quadrant of right orbit; with limited abduction in the right eye [Figure 1]. She gave history of mass excision done twice in the past elsewhere. Magnetic resonance imaging orbit revealed a well-defined oval lesion measuring 2.4 cm × 1.6 cm in the inferolateral aspect of right orbit extending from the rim to just behind the globe causing mild indentation and displacement of the globe [Figure 2]. It was isointense on T1W1 and mixed hypo and hyperintense signal in T2W1 along with central low signal areas. The lateral rectus and inferior oblique muscles were involved. Patient underwent an incisional biopsy of the lesion via inferior forniceal approach and was found to have a pale gritty mass; separate from the muscles and abutting the globe. Histopathological examination revealed fibrocollagenous tissue with perivascular exudates of eosinophils accompanied by collection of plasma cells and lymphocytes. There was absence of any fibrinoid necrosis. Extensive areas of perivascular fibrosis showing a characteristic angiocentric whirling with an onion skin pattern was identified [Figure 3] and [Figure 4]. Special stain for fungi was negative. This suggested the diagnosis of eosoinophilic angiocentric fibrosis. She had no significant medical history and no nasal, sinus, or atopic symptoms. There was no history of either drug or environmental allergies. Laboratory investigations revealed raised erythrocyte sedimentation rate, however complete blood count was normal, with no peripheral eosinophilia. Autoimmune screening including antinuclear antibody, antineutrophil cytoplasmic antibody (ANCA) and rheumatoid factor was negative. ENT review showed absence of any nasal and upper respiratory pathology. She underwent surgical debulking of the mass via inferior orbitotomy. Postoperatively, she was started on oral prednisolone 60 mg (1 mg/kg body weight), which was tapered over 3 months. At her last follow-up visit, she was completely free of orbital disease.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

 DISCUSSION



Eosinophilic angiocentric fibrosis is a rare clinical entity characterized by an indolent, but the progressive, inflammatory process of the upper respiratory mucosa with peculiar histopathological findings. It was first described by Holmes and Panje [1] in 1983 and further characterized by Roberts and McCann [2] in 1985. The exact pathophysiology of EAF remains unclear. Some authors suggest that the association with atopic disorders and the characteristic eosinophilic infiltrate on histopathology may represent an abnormal inflammatory response to nonspecific stimulus in predisposed individuals. Recently, Deshpande et al. [7] suggested that EAF is a form of IgG4-related systemic disease (RSD) and bear several hallmarks of IgG4-RSD, including the presence of a tumefactive lesion, elevated numbers of IgG4-bearing plasma cells, and an elevated lesional IgG4: IgG ratio.

Most patients present with nonspecific nasal or sinus related symptoms with generally a long delay in the diagnosis. The rare ophthalmic manifestations of this disease include epiphora, diplopia, dystopia and proptosis. [4],[5],[6],[7],[8] Orbital involvement by EAF first was reported by Paun et al., [3] who described three patients with extensive disease centered on the nasal cavity and had widespread infiltration of the facial soft tissues and orbits. In one patient, the orbital component appeared only later in the disease process, and in two cases, it was part of the recurrence of disease after several radical attempts at surgical clearance. Since then, only six cases of primary orbital disease have been described in the literature [Table 1].{Table 1}

Magnetic resonance imaging studies have shown an isointense orbital mass on T1W scans in close proximity to the paranasal sinuses and associated sinus mucosal thickening. Middle ethmoidal air cells seem to be predisposed. [4],[5],[8] Typical histopathological features are the basis for establishing the diagnosis. [2] Histologically the lesion can be separated into early and late stages, which could both be seen in a single biopsy. The early phase consists of an eosinophilic vasculitis without fibrinoid necrosis, occurring in a patchy fashion and involving groups of capillaries and/or venules. Eosinophils cluster and migrate through the vessel wall and are accompanied by a mixture of plasma cells, lymphocytes, and fibroblasts. The arterioles and small arteries are not involved. A pseudogranulomatous reaction is often seen, but there are no epithelioid cells or multinucleated giant cells which differentiates this condition from the inflammatory diseases like Wegner's granulomatois (WG). The late lesion is characterized by a dense fibrous thickening of the stroma and a characteristic obliterative perivascular onion skin whorling of collagen fibers and reticulin. The inflammatory infiltrate becomes progressively hypocellular with increasing fibrosis, and eventually only eosinophils remain. These typical histological findings with both early and late phases of the disease were noted in our patient too.

The differential diagnosis of EAF includes WG, Churg-Strauss syndrome, sarcoidosis, Sjögren's disease, Kimura's disease, erythema elevatum diutinum, granuloma faciale, and certain tumors such as neurogenic tumors (schwannoma), vascular tumors (angiofibroma, hemangiomas), and mesenchymal tumors (fibroma, fibrosarcoma, nodular fasciitis). [9] The lack of systemic signs and symptoms, negative autoimmune serology and lack of granulomas on histology helps to exclude majority of the inflammatory diseases. However, limited form of WG may present a diagnostic challenge in certain cases. In both cases, ANCA may be negative and eosinophils may predominate on histopathology. Although eosinophils and fibrosis can be seen in early cases of orbital WG, the well-developed angiocentric pattern of fibrosis in our case suggests a well-established chronic lesion, and more typical changes of WG like necrotizing granulomatous vasculitis would be expected to appear by then. [10]

Granuloma faciale is an uncommon disease characterized by sharply circumscribed skin plaques or nodules on the face. Some authors consider EAF as an extracutaneous manifestation of granuloma faciale. Plaque - like lesions of the oral mucosa and upper airways have been described in this condition. [11]

Once the diagnosis of EAF is established, the treatment options include systemic immunosuppressive agents or surgical debulking of the orbital mass lesion. Attempts to decrease the inflammatory process with systemic steroids have been largely disappointing. Other immunosuppressive agents such as dapsone, azathioprine, and hydroxychloroquine have been tried with limited success. Surgical excision is the treatment of choice. However, recurrences are not uncommon and may require repeated intervention.

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