Eosinophilic angiocentric fibrosis M31.3

Holmes and Panje 1983

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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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).

Middle adulthood between 30-60 years

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.

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

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.

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

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
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7. Nigar E et al (2007) Eosinophilic angiocentric fibrosis and extrafacial granuloma faciale. Histopathology 51: 729-731.
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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.
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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.