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Ocular Juvenile Xanthogranuloma in an Older Teenager

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 19-year-old man presented with recurrent left eye redness and photophobia for the past year. The patient had previously been treated with topical corticosteroid therapy, which provided temporary relief of his symptoms. Visual acuity was 20/20 OU with normal intraocular pressures. The results from the slitlamp examination of the right eye were within normal limits (Figure 1A). Examination results of the left eye demonstrated 2+ diffuse conjunctival hyperemia, 1+ anterior chamber cell, and a vascularized iris infiltrate (Figure 1B). Results of the dilated fundus examination were within normal limits in both eyes. Ultrasound biomicroscopy of the left eye demonstrated areas of ciliary body thickening 360° and a thickened iris pupillary margin superiorly and nasally. A full review of systems was negative for other abnormalities including skin lesions. Test results for tuberculosis and syphilis were negative.

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Juvenile xanthogranuloma

A. Perform biopsy of the iris lesion

The patient had a unilateral iris infiltrate with recurrent inflammation that was responsive to topical corticosteroid therapy. The differential diagnosis included juvenile xanthogranuloma (JXG), lymphoma, leukemia, sarcoidosis, HLA-B27–associated uveitis, syphilis, and tuberculosis.

Of the options above, a biopsy of the lesion seemed most likely to determine the cause and best future treatment modality (choice A). Administration of a subtenon corticosteroid injection (choice B) may be useful in the treatment of inflammation once a diagnosis is made. An anterior chamber (AC) paracentesis for viral polymerase chain reaction (choice C) is unlikely to be helpful, as one would not expect an infiltrative process in a viral uveitis. Screening for lupus with antinuclear antibody testing (choice D) would not be helpful because the presentation as described is not a manifestation of lupus, nor does lupus cause uvetis. Testing for systemic causes such as HLA-B27, syphilis, tuberculosis, lymphoma, leukemia, and sarcoidosis should also be performed but will be less likely to yield a final diagnosis when compared with biopsy results of involved tissue.

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Article Information

Corresponding Author: Eric L. Crowell, MD, MPH, Ruiz Department of Ophthalmology and Visual Science, McGovern Medical School, 6400 Fannin St, Ste 1800, Houston, TX 77030 (eric.l.crowell@utexas.edu).

Published Online: January 30, 2020. doi:10.1001/jamaophthalmol.2019.4982

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Zimmerman  LE.  Ocular lesions of juvenile xanthogranuloma: nevoxanthoedothelioma.  Am J Ophthalmol. 1965;60(6):1011-1035. doi:10.1016/0002-9394(65)92808-4PubMedGoogle ScholarCrossref
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Weitzman  S, Jaffe  R.  Uncommon histiocytic disorders: the non–Langerhans cell histiocytoses.  Pediatr Blood Cancer. 2005;45(3):256-264. doi:10.1002/pbc.20246PubMedGoogle ScholarCrossref
3.
Hernandez-Martin  A, Baselga  E, Drolet  BA, Esterly  NB.  Juvenile xanthogranuloma.  J Am Acad Dermatol. 1997;36(3, pt 1):355-367. doi:10.1016/S0190-9622(97)80207-1PubMedGoogle ScholarCrossref
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Chang  MW, Frieden  IJ, Good  W.  The risk intraocular juvenile xanthogranuloma: survey of current practices and assessment of risk.  J Am Acad Dermatol. 1996;34(3):445-449. doi:10.1016/S0190-9622(96)90437-5PubMedGoogle ScholarCrossref
5.
Freyer  DR, Kennedy  R, Bostrom  BC, Kohut  G, Dehner  LP.  Juvenile xanthogranuloma: forms of systemic disease and their clinical implications.  J Pediatr. 1996;129(2):227-237. doi:10.1016/S0022-3476(96)70247-0PubMedGoogle ScholarCrossref
6.
Samara  WA, Khoo  CTL, Say  EAT,  et al.  Juvenile xanthogranuloma involving the eye and ocular adnexa: tumor control, visual outcomes, and globe salvage in 30 patients.  Ophthalmology. 2015;122(10):2130-2138. doi:10.1016/j.ophtha.2015.06.009PubMedGoogle ScholarCrossref
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