International Ophthalmology

, Volume 31, Issue 3, pp 245–248 | Cite as

Xanthogranulomatous variant of immunoglobulin G4 sclerosing disease presenting as ptosis, proptosis and eyelid skin plaques

  • Hardeep S. Mudhar
  • Rina Bhatt
  • Soupramanien Sandramouli
Case Report


A 70-year-old male was referred to the oculoplastic clinic with left-sided ptosis and floppy eyelids. During follow-up, bilateral upper lid xanthelasma developed with worsening ptosis and proptosis, which was worse on the left side. A left orbital biopsy showed xanthogranulomatous inflammation of the orbit. The patient was treated with a variety of immune modulator regimes but due to a variety of side-effects, treatment was discontinued. The left orbit was surgically debulked twice and histology revealed xanthogranulomatous inflammation, with the additional features of sclerosis, lymphoid aggregates and a prominent population of plasma cells. Around 80% of the plasma cells expressed immunoglobulin G4 (IgG4). This case report reveals an association between xanthogranulomatous inflammation of the orbit and a prominent population of IgG4-positive plasma cells. We propose that the overall disease is a novel variant of IgG4 sclerosing disease of the orbit and suggest that cases of histologically proven xanthogranulomatous inflammation should be stained for IgG4 if there is an accompanying plasma cell population.


IgG4 Xanthogranulomatous inflammation Sclerosing inflammation 


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Copyright information

© Springer Science+Business Media B.V. 2011

Authors and Affiliations

  • Hardeep S. Mudhar
    • 1
  • Rina Bhatt
    • 2
  • Soupramanien Sandramouli
    • 2
  1. 1.Department of HistopathologyNational Specialist Ophthalmic Pathology Service (NSOPS-Sheffield Lab)SheffieldUK
  2. 2.Wolverhampton and Midland Counties Eye Infirmary, New Cross HospitalWolverhamptonUK

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