Eosinophilic angiocentric fibrosis

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Synonyms: IgG4-related eosinophilic angiocentric fibrosis

A rare otorhinolaryngologic disease characterized by an indolent submucosal mass of variable size and extent most commonly arising in the anterior nasal cavity involving the nasal septum and lateral nasal wall and potentially extending into the adjacent sinuses. Occurrence in the larynx and lower respiratory tract or the orbit is rare. Histological examination shows concentric angiocentric stromal fibrosis (onionskin fibrosis) and prominent eosinophils. Increased numbers of IgG4-positive plasma cells in the lesion may also be observed in addition to elevated serum IgG4. Patients typically present with long-standing obstructive symptoms.

Data from Orphanet are used to provide information on a disease's name, synonym(s), and overview. Reference: Access aggregated data from Orphanet at Orphadata. Orphadata: Free access data from Orphanet. © INSERM 1999. Available on http://www.orphadata.org. Data version July 2024

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