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

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.

The Primary Brain Eosinophilic Angiocentric Fibrosis, A Rare Case Report

“…Eosinophilic angiocentric fibrosis (EAF) is a rare progressive fibrosing lesion involving the nasal cavity, paranasal sinuses, and the upper respiratory tract (Ahn and Flanagan, 2018). There are few reports that it rarely involves the orbit; however, there is no report of intracranial involvement (Radhakrishnan et al, 2015;Ahn and Flanagan, 2018). Here, we report and share our experience with a rare case of primary intracranial EAF.…”

“…In addition, there are reports of its association with the granuloma faciale and granulomatosis with polyangiitis (GPA) (previously Wegener's granulomatosis). Furthermore, some maintain that it is a progressive fibrotic reaction rather than a true separate disease (Lloyd et al, 2015;Radhakrishnan et al, 2015;Ahn and Flanagan, 2018).…”

“…The diagnostic histologic features for IgG-RD include morphologic appearance, storiform type fibrosis, increased number of IgG4 plasma cells (more than 50 cells in each high-power field), and a ratio of plasmacells IgG4/IgG more than 40%. In this situation, other pathologies associated with increased IgG should be evaluated and rolled out, such as primary sclerosing cholangitis, rheumatoid arthritis, and lymphoma (Radhakrishnan et al, 2015;Ahn and Flanagan, 2018).…”

“…These include reactive conditions (Wegener's granulomatosis, sarcoidosis, Sjogren's disease, Kimura's disease, erythema elevatum diutinum, granuloma faciale, granulomatosis with polyangiitis, Churg-Strauss syndrome, and angio lymphoid hyperplasia with eosinophilia), neoplastic lesions, such as neurogenic tumours (schwannoma), vascular tumours (angiofibroma, hemangiomas), and mesenchymal tumours (fibroma, fibrosarcoma, nodular fasciitis, and fibromatosis). Many of these diseases can be ruled out by using history and physical examination, autoimmune serology and other laboratory data, as well as the absence of granuloma on histology (Radhakrishnan et al, 2015;Ahn and Flanagan, 2018).…”

“…Recently, some authors considered the relation between EAF and IgG-RD, and some explained that EAF might be a type of IgG-related disease (Deshpande et al, 2011;Gallo et al, 2017;Ahn and Flanagan, 2018). Diagnostic criteria for IgG4-RD include histologic finding (as noted earlier), presence of a tumefactive lesion, multiple classic organ involvement, subacute onset, elevated serum IgG4, and plasma cells, and rapid response to immunosuppressive treatment (Radhakrishnan et al, 2015;Ahn and Flanagan, 2018).…”