Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence

Aleza A. Andron, Akshay Gopinathan Nair, David Della Rocca, Robert C. Della Rocca, Harsha S. Reddy

Research output: Contribution to journalArticlepeer-review

5 Scopus citations


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.

Original languageEnglish
Pages (from-to)108-111
Number of pages4
Issue number1
StatePublished - 2022
Externally publishedYes


  • IgG-4 ROD
  • lacrimal gland
  • orbit
  • xanthogranuloma


Dive into the research topics of 'Concomitant adult onset xanthogranuloma and IgG4-related orbital disease: a rare occurrence'. Together they form a unique fingerprint.

Cite this