Published date: Jun 21 2024

Journal Title: Journal of Ophthalmic and Vision Research

Issue title: Apr–Jun 2024, Volume 19, Issue 2

Pages: 255–259

DOI: 10.18502/jovr.v19i2.9629

Sahba Fekri - fekri22sahba@yahoo.com - https://orcid.org/0000-0002-7388-6725

Mohamad Hasan Rikhtehgar - hasan_794@yahoo.com

Abbas Bagheri - abbasbagheri@yahoo.com - https://orcid.org/0000-0002-6736-7435

Amirreza Veisi - amirveisi3@gmail.com - https://orcid.org/0000-0001-8304-3804

Amir Akhavan Azari - amirazarimd@gmail.com

Purpose: To report a case of adult-onset asthma and periocular xanthogranuloma (AAPOX) in a patient with intermediate uveitis and a history of Hodgkin’s lymphoma (HL).

Case Report: A 51-year-old man with a past medical history of HL presented with blurred vision, asthma, and bilateral yellowish eyelid lesions. The eyelid lesions and asthma appeared 10 years after being diagnosed with HL. Physical examination was significant for multiple subcutaneous and firm eyelid masses in addition to the presence of pre-auricular and submandibular lymphadenopathies. Ophthalmic examination revealed bilateral intermediate uveitis and mild macular edema. Further systemic evaluations, including laboratory testing and imaging, were normal. Excisional biopsy of the eyelid lesions was performed and the histopathologic examination was consistent with the diagnosis of AAPOX.

Conclusion: The presence of AAPOX in a patient with intermediate uveitis and history of HL suggests that immunological dysfunction may play a role in the pathogenesis of adult orbital xanthogranulomatous disease.

1. Kerstetter J, Wang J. Adult orbital xanthogranulomatous disease: A review with emphasis on etiology, systemic associations, diagnostic tools, and treatment. Dermatol Clin 2015;33:457–463.

2. Jakobiec FA, Mills MD, Hidayat AA, Dallow RL, Townsend DJ, Brinker EA, et al. Periocular xanthogranulomas associated with severe adult-onset asthma. Trans Am Ophthalmol Soc 1993;91:99–125.

3. Sivak-Callcott JA, Rootman J, Rasmussen SL, Nugent RA, White VA, Paridaens D, et al. Adult xanthogranulomatous disease of the orbit and ocular adnexa: new immunohistochemical findings and clinical review. Br J Ophthalmol 2006;90:602–608.

4. Guo J, Wang J. Adult orbital xanthogranulomatous disease: Review of the literature. Arch Pathol Lab Med 2009;133:1994–1997.

5. Shams PN, Rasmussen SL, Dolman PJ. Adult-onset asthma associated with simultaneous conjunctival, eyelid, and orbital xanthogranulomatosis responsive to systemic immunosuppression. Ophthalmic Plast Reconstr Surg 2015;31:e162–3.

6. Hammond MD, Niemi EW, Ward TP, Eiseman AS. Adult orbital xanthogranuloma with associated adult-onset asthma. Ophthalmic Plast Reconstr Surg 2004;20:329– 332.

7. London J, Martin A, Soussan M, Badelon I, Gille T, Uzunhan Y, et al. Adult Onset Asthma and Periocular Xanthogranuloma (AAPOX), a rare entity with a strong link to IgG4-related disease: An observational case report study. Medicine 2015;94:e1916.

8. Stelmach-Gołdyś A, Garus B, Skowronek M, Kopczyński J, Trzpiot S, Pasiarski M. Periocular xanthogranuloma with adult-onset asthma: a rare disorder associated with elevated IgG4 levels. Pol Arch Intern Med 2019;129:827– 829.

9. Roggin KK, Rudloff U, Klimstra DS, Russell LA, Blumgart LH. Adult-onset asthma and periocular xanthogranulomas in a patient with lymphoplasmacytic sclerosing pancreatitis. Pancreas 2007;34:157–160.