Eosinophilic angiocentric fibrosis M31.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Eosinophilic Angiocentric Fibrosis

History
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Holmes and Panje 1983

Definition
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Rare benign disease of unknown aetiology, which may affect the sinonasal tract and the upper respiratory tract, more rarely the orbit. The disease is interpreted as a (monotopic) site-varying manifestation of an IgG4-associated autoimmune disease.

Concordant occurrence with the facial granuloma (eosinophlicum) has been observed several times (Burns BV et al. 2001; Yung A et al. 2005)

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Occurrence/Epidemiology
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Etiopathogenesis
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Unknown, type I sensitizations (e.g. mold allergy, house dust or plant pollen) are frequently detected. In one case a cocaine abus with a nasal septum perforation was present. Since eosinophilic angiocentric fibrosis belongs to the family of IgG4-associated autoimmune diseases, the pathomechaním is likely to be similar, although with organ-related variations (see there).

Manifestation
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Middle adulthood between 30-60 years

Clinical features
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There are 2.0-5.0 cm large, "pseudotumorous" submucous, firm swellings which can lead to an increasing nasal obstruction. Often epistaxis (may be the first clinical sign of the disease). The radiological findings are non-specific and show an expanding soft tissue tumor gfls. also with bone erosion.

Laboratory
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Increased serum IgG4 concentrations are detectable (as with other IgG4-associated diseases).

Histology
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Detection of a perivascular eosinophilic infiltrate with lymphocytes and plasma cells as well as with conspicuous accompanying perivascular fibrosis which can lead to a characteristic onion-skin-like aspect.

Therapy
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Systemic steroidal therapy with subsequent surgical rehabilitation. A high local recurrence rate must be expected.

Literature
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  1. Chen VH et al (2016) A Concomitant Case of Orbital Granuloma Faciale and Eosinophilic Angiocentric Fibrosis. Ophthal Plast Reconstr Surg PubMed PMID: 27281484.
  2. Burns BV et al (2001) Eosinophilic angiocentric fibrosis affecting the nasal cavity. A mucosal variant of the skin lesion granuloma faciale. J Laryngol Otol 115:223-226.
  3. Chinelli PA et al (2004) Granuloma faciale associated with sinonasal tract eosinophilic angiocentric fibrosis. Acta Derm Venereol 84:486-487.
  4. Holme SA et al (2005) Concurrent granuloma faciale and eosinophilic angiocentric fibrosis. Br J Dermatol 153: 851-853 Jain R et al (2008)
  5. Holmes DK et al (1983) Intranasal granuloma facial. At J Otolaryngol 4: 184-186
  6. Narayan J et al (2005) Eosinophilic angiocentric fibrosis and granuloma faciale: analysis of cellular infiltrate and review of literature. Ann Otol Rhinol Laryngol 114: 35-42.
  7. Nigar E et al (2007) Eosinophilic angiocentric fibrosis and extrafacial granuloma faciale. Histopathology 51: 729-731.
  8. Nogueira A et al (2011) Granuloma faciale with subglottic eosinophilic angiocentric fibrosis: case report and review of the literature. Cutis 88:77-82.
  9. Singh A et al (2015) Eosinophilic Angiocentric Fibrosis of Sinonasal Region: A Rare & Under Reported Entity. J Clin Diagn Res 9: ED 05-6.
  10. Stelini RF et al (2016) Granuloma Faciale and Eosinophilic Angiocentric Fibrosis: Similar Entities in Different Anatomic Sites. Appl Immunohistochem Mol Morphol PubMed PMID: 26808128.
  11. Vassallo C et al (2015) Chronic localized leukocytoclastic vasculitis: clinicopathological spectrum of granuloma faciale with and without extrafacial and mucosal involvement. G Ital Dermatol Venereol 150:87-94.
  12. Watanabe N et al (2005) Atypical eosinophilic angiocentric fibrosis on nasal septum. Auris nasal larynx 33:355-358.
  13. Yung A et al (2005) Eosinophilic angiocentric fibrosis--a rare mucosal variant of granuloma faciale which may present to the dermatologist. Br J Dermatol 152: 574-576.
  14. Roberts PF et al (1985) Eosinophilic angiocentric fibrosis of the upper respiratory tract: a mucosal variant of granuloma faciale? A report of three cases. Histopathology 9: 1217-1225.

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020