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

Abstract

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.

Keywords

IgG4 Xanthogranulomatous inflammation Sclerosing inflammation 

References

  1. 1.
    Sivak-Callcott JA, Rootman J, Rasmussen SL, Nugent RA et al (2006) Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review. Br J Ophthalmol 90(5):602–608 (Review)PubMedCrossRefGoogle Scholar
  2. 2.
    Kamisawa T, Okomoto A (2008) IgG4-related sclerosing disease. World J Gastroenterol 14:3948–3955PubMedCrossRefGoogle Scholar
  3. 3.
    Takahira M, Kawano M, Zen Y, Minato H, Yamada K, Sugiyama K (2007) IgG4-related chronic sclerosing dacryoadenitis. Arch Ophthalmol 125(11):1575–1578Google Scholar
  4. 4.
    Cheuk W, Yuen HK, Chan JK (2007) Chronic sclerosing dacryoadenitis: part of the spectrum of IgG4-related sclerosing disease? Am J Surg Pathol 31(4):643–645PubMedCrossRefGoogle Scholar
  5. 5.
    Kubota T, Moritani S, Katayama M, Terasaki H (2010) Ocular adnexal IgG4-related lymphoplasmacytic infiltrative disorder. Arch Ophthalmol 128(5):577–584PubMedCrossRefGoogle Scholar
  6. 6.
    Cheuk W, Yuen HK, Chan AC, Shih LY, Kuo TT, Ma MW, Lo YF, Chan WK, Chan JK (2008) Ocular adnexal lymphoma associated with IgG4+ chronic sclerosing dacryoadenitis: a previously undescribed complication of IgG4-related sclerosing disease. Am J Surg Pathol 32:1159–1167PubMedCrossRefGoogle Scholar
  7. 7.
    Cotran RS, Kumar V, Collins T (eds) (1999) Robbins pathologic basis of disease, Chap 23, 6th edn. WB Saunders, Philadelphia, p 1126Google Scholar
  8. 8.
    Kuo TT, Chen TC, Lee LY, Lu PH (2009) IgG4-positive plasma cells in cutaneous Rosai–Dorfman disease: an additional immunohistochemical feature, possible relationship to IgG4-related sclerosing disease. J Cutan Pathol 36(10):1069–1073PubMedCrossRefGoogle Scholar
  9. 9.
    Roberts SS, Attanoos RL (2010) IgG4+ Rosai–Dorfman disease of the lung. Histopathology 56(5):662–664PubMedCrossRefGoogle Scholar
  10. 10.
    Sivak-Callcott JA, Rootman J, Rasmussen SL, Nugent RA, White VA, Paridaens D, Currie Z, Rose G, Clark B, McNab AA, Buffam FV, Neigel JM, Kazim M (2006) Adult xanthogranulomatous disease of the orbit, ocular adnexa: new immunohistochemical findings, clinical review. Br J Ophthalmol 90(5):602–608PubMedCrossRefGoogle Scholar

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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