Open AccessEosinophilic angiocentric fibrosis : a sino-orbital masquerader
Author(s) -
Nicole Legare,
Seema Frosh,
Jonathan Vásquez,
Son Ho
Publication year - 2018
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr-2017-223675
Subject(s) - medicine , granulomatosis with polyangiitis , eosinophilic , pathology , anti neutrophil cytoplasmic antibody , fibrosis , igg4 related disease , lung , soft tissue , pulmonary fibrosis , vasculitis , disease
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.