Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: A rare occurrence Journal Article


Authors: Andron, A. A.; Nair, A. G.; Della Rocca, D.; Della Rocca, R. C.; Reddy, H. S.
Article Title: Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: A rare occurrence
Abstract: Adult onset xanthogranulomatous disease is a rare orbital disease. IgG4-related disease is a systemic disease that can often manifest in the orbit. In this communication, we present the case of a patient with a xanthelasma-like lesion on the upper eyelid, and an enlarged lacrimal gland, which on biopsy was diagnosed as an orbital xanthogranuloma. Detailed serological workup showed that the patient was found to have elevated serum IgG4 levels. The orbital specimen was re-stained and found to be positive for IgG4. The patient was treated with oral steroids with partial resolution of the lesion. This is an interesting case of both histopathological adult onset xanthogranuloma (AOX) and IgG4-related orbital disease (IgG4-ROD). The early evidence suggests that the diagnosis of one of these disorders should point the physician to investigate for the presence of the other, especially if xanthogranulomatous disease is diagnosed first. © 2020 Taylor & Francis Group, LLC.
Keywords: adult; case report; immunoglobulin g; xanthogranuloma; granuloma; orbit; lacrimal gland; orbit disease; orbital diseases; xanthomatosis; humans; human; igg-4 rod; immunoglobulin g4-related disease
Journal Title: Orbit
Volume: 41
Issue: 1
ISSN: 0167-6830
Publisher: Taylor & Francis Group  
Date Published: 2022-01-01
Start Page: 108
End Page: 111
Language: English
DOI: 10.1080/01676830.2020.1814353
PUBMED: 32847452
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 1 March 2022 -- Source: Scopus
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