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Necrobiotic xanthogranuloma and chronic lymphocytic leukemia of the conjunctiva masquerading as scleritis and uveitis

Authors Mohsenin A, Sinard, Huang

Received 8 July 2012

Accepted for publication 23 August 2012

Published 13 December 2012 Volume 2012:6 Pages 2045—2047

DOI https://doi.org/10.2147/OPTH.S35743

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 3


Amir Mohsenin,1 John Sinard,1,2 John J Huang1

1Department of Ophthalmology and Visual Science, 2Department of Pathology, Yale University School of Medicine, New Haven, CT, USA

Abstract: This report describes a unique case of coexisting necrobiotic xanthogranuloma and chronic lymphocytic leukemia in a patient presenting with scleritis and uveitis. A 53-year-old Caucasian man diagnosed with anterior uveitis and scleritis for the prior year was referred to our uveitis clinic for further evaluation. Prior uveitis/scleritis workup performed by the referring ophthalmologist was negative. Examination demonstrated unilateral uveitis and posterior scleritis along with bilateral conjunctival lesions. Incisional biopsy of the conjunctiva was carried out. The excised tissue was sent fresh for histopathologic studies. Slit-lamp examination revealed bilateral yellow-white bulbar conjunctival lesions, 1+ conjunctival injection OU, stellate keratic precipitates OS with 25 cells per high-powered field. Funduscopic examination was significant for subretinal fluid OS. Conjunctival biopsy revealed necrobiotic xanthogranuloma and chronic lymphocytic leukemia. Systemic workup demonstrated paraproteinemia consistent with necrobiotic xanthogranuloma and a complete blood count consistent with leukemia. This case demonstrates an atypical presentation of necrobiotic xanthogranuloma and concomitant chronic lymphocytic leukemia presenting in the form of uveitis, scleritis, and conjunctival infiltration. Masquerade syndromes, such as necrobiotic xanthogranuloma and leukemia, must be kept in mind when treating patients with uveitis or scleritis with negative workups.

Keywords: necrobiotic xanthogranuloma, chronic lymphocytic leukemia, uveitis, scleritis, masquerade syndrome

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