Abstract:

Eosinophilic angiocentric fibrosis (EAF) is a rare tumefactive lesion demonstrating indolent behavior with involvement of the sinonasal cavity, orbit, and respiratory tract. A spectrum of histologic findings may include prominent eosinophils, lymphoplasmacytic inflammation, and concentric angiocentric fibrosis (onionskin fibrosis). Occasionally, lymphoid follicles may be appreciated. The pathogenesis of EAF is poorly understood, however, recent reports place it on a possible continuum of immunoglobulin G4 (IgG4)-related disease. The characterization of EAF continues to evolve as its recognition increases in the literature. We present a case of a 44-year-old woman who presented with a non-tender, enlarging left superior orbital mass. The patient reported being unable to fully open the left eye but had no visual impairment. Histopathologic evaluation following biopsy of the left lacrimal gland showed onion-skinning fibrosis concentrically around small vessels, prominent plasma cells and scattered eosinophils, consistent with EAF. Immunohistochemistry staining for total IgG and IgG4 revealed an IgG4/IgG ratio of approximately 5%. Quantitative serum levels of IgG4 were within normal limits. The patient subsequently underwent surgical excision, and histopathologic evaluation demonstrated similar angiocentric onion-skinning fibrosis without the presence of IgG4 positive cells by immunohistochemistry. Thus, our case of EAF did not meet diagnostic criteria for IgG4-related disease. It is our aim to add to the paucity of data evaluating EAF as an IgG4-related disease by highlighting a rare case of orbital EAF.

Financial Disclosure:
No current or relevant financial relationships exist.

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Hannah Clark, BS