Eosinophilic angiocentric fibrosis : a sino-orbital masquerader

Abstract

A 58-year-old Caucasian male presented with left periorbital oedema extending to the nasal area for 1 year along with nasal discharge for 1 month. Lab work was significant for positive cytoplasmic antineutrophil cytoplasmic antibodies. CT scan showed solid mass along the nasal soft tissue with bony nasal destruction. A CT scan of the thorax was performed to rule out granulomatosis with polyangiitis and showed multiple pulmonary nodules. Biopsies of the nasal mass and lung nodule were performed which showed fragments of fibrosis with spindle cell proliferation consistent with eosinophilic angiocentric fibrosis (EAF). EAF is a very rare disease, recently described as a subtype of immunoglobulin G4-related disease. A few rare cases of EAF involving the structures of the orbit have been reported in the literature.

Keywords: ophthalmology; rheumatology.

Figure 1

Axial CT scan of the orbits illustrating a solid mass along the nasal soft tissue extending bilaterally into the medial periorbital soft tissue. Bony nasal destruction is noted extending into the mid/superior nasal septum and anterior nasal cavity mucosa.

Figure 2

(A) Photomicrograph of the orbital mass showing spindle cell proliferation and fibrosis associated with mixed inflammatory cells (H&E 40×).(B) Lung nodule displaying fibrosis with spindle cell proliferation and mixed inflammatory infiltrate consisting of lymphocytes with no atypia, plasma cells, eosinophils and neutrophils (H&E 40×).