Proper ethmoid eosinophilic angiocentric fibrosis with orbital extension.
Eur Ann Otorhinolaryngol Head Neck Dis. 2017 Oct;134(5):351-354
Authors: Gorostis S, Bacha M, Gravier S, Raguin T
INTRODUCTION: Eosinophilic angiocentric fibrosis (EAF) is a slowly progressive, benign illness involving the mucosa of the higher airways or, extra not often, the orbit. It belongs to the spectrum of IgG4-related illness.
CASE REPORT: The authors report the case of a 61-year-old man who introduced with orbital involvement (visible loss, ache, proptosis, and eyelid oedema), headache and nasal obstruction. Imaging revealed a proper ethmoido-orbital mass infiltrating the periorbital fats and enveloping the optic nerve. Histological examination concluded on a analysis of EAF within the presence of perivascular infiltration by inflammatory cells, predominantly eosinophils, and zones of “onion pores and skin” fibrosis. Immunohistochemistry attributed these lesions to IgG4-related illness. Preliminary remedy with corticosteroids adopted by dapsone failed to regulate the illness and resulted in extreme steroid dependence. Surgical ethmoidectomy with resection of the lamina papyracea was carried out to permit displacement of the eyeball into the nasal cavity within the occasion of one other episode.
DISCUSSION: The mixture of surgical procedure and rituximab achieved lasting ache reduction with no recurrence of exophthalmos.
PMID: 28359733 [PubMed – indexed for MEDLINE]