Orbital necrobiotic xanthogranuloma associated with systemic IgG4 disease.

Abstract

PURPOSE:

The authors describe 2 cases of orbital xanthogranulomatous disease associated with an increase in IgG4-positive plasma cells, and also examine IgG4 in other types of orbital inflammation.

METHODS:

Immunohistochemistry for total IgG and IgG4 was performed in 18 cases of orbital inflammation, including chronic dacryoadenitis (n=10), necrobiotic xanthogranuloma (n=2), xanthogranuloma (n=1), idiopathic orbital inflammation/pseudotumor (n=4), and fungal infection (n=1).

RESULTS:

One patient presenting with necrobiotic xanthogranuloma had signs of systemic IgG4 disease. His orbital lesion showed an elevated number of IgG4 positive plasma cells (55%). An orbital xanthogranulomatous lesion in a second patient lacking systemic symptoms also contained a high percentage of IgG4-positive plasma cells (80%). Only 1 case of chronic dacryoadenitis contained prominent IgG4-positive plasma cells (mean 17/hpf).

CONCLUSIONS:

IgG4-positive plasma cells are relatively rare in nonsclerosing orbital inflammatory lesions. However, systemic disease IgG4 can be associated with necrobiotic xanthogranuloma of the orbit.

• Authors: Kamaljeet Singh, K D A Rajan, Charles Eberhart
• Journal: Ocular immunology and inflammation (Ocul Immunol Inflamm) Vol. 18 Issue 5 Pg. 373-8 (Oct 2010) ISSN: 1744-5078 [Electronic] England
• PMID: 20716008 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin G

Topics

• Adolescent
• Adult
• Aged
• Aged, 80 and over
• Child
• Chronic Disease
• Dacryocystitis (immunology, pathology)
• Female
• Humans
• Immunoglobulin G (metabolism)
• Immunohistochemistry
• Inflammation (immunology, pathology)
• Lymphocytes (pathology)
• Male
• Middle Aged
• Necrobiotic Xanthogranuloma (immunology, pathology)
• Orbital Diseases (immunology, pathology)
• Pancreatitis (immunology, pathology)
• Plasma Cells (metabolism)
• Sclerosis