title: [Eosinophilic angiocentric fibrosis: a form of IgG4-related systemic disease?].
abstract: Eosinophilic angiocentric fibrosis is a rare fibro-inflammatory disorder of unknown etiology with only 40 cases reported in the literature. It primarily affects the sinonasal tract and more rarely the orbit, the larynx and the gums. This benign disorder is characterized by a slowly progressive process mimicking a tumor, with frequent recurrences after surgical excision and cortico-therapy. The typical histology consists of fibro-inflammatory lesion with numerous eosinophils, arranged in a perivascular pattern. As the lesion matures, inflammation becomes less intense and the fibrosis progresses with an onion-skin type perivascular fibrosis. A recent paper suggests that EAF is part of the spectrum of IgG4-related systemic disease. We report a case of orbital EAF in an 86-year-old woman which sustained this hypothesis.
authors.affiliation: Département de pathologie, université Montpellier 1, hôpital Gui-de-Chauliac, CHU de Montpellier, 80, avenue A-Fliche, 34295 Montpellier cedex 5, France.
authors.forename: Amal
authors.initials: A
authors.lastname: Benlemlih
authors.forename: Vanessa
authors.initials: V
authors.lastname: Szableski
authors.forename: Mouhcine
authors.initials: M
authors.lastname: Bendahou
authors.forename: Sophie
authors.initials: S
authors.lastname: Rivière
authors.forename: Max
authors.initials: M
authors.lastname: Villain
authors.forename: Valérie
authors.initials: V
authors.lastname: Costes
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000369:Aged, 80 and over; D001402:B-Lymphocytes / Q000473:pathology; D004172:Diplopia / Q000209:etiology; D018450:Disease Progression; D004802:Eosinophilia / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D005094:Exophthalmos / Q000209:etiology; D005260:Female; D005355:Fibrosis; D006801:Humans; D006942:Hypergammaglobulinemia / Q000145:classification*; D007074:Immunoglobulin G / Q000276:immunology*; D008279:Magnetic Resonance Imaging; D009915:Orbit / Q000098:blood supply; D009916:Orbital Diseases / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D010950:Plasma Cells / Q000276:immunology / Q000473:pathology; D013601:T-Lymphocytes / Q000473:pathology; D014786:Vision Disorders / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones; D007074:Immunoglobulin G