Abstract | November 17, 2023

Necrobiotic Xanthogranuloma Associated with Uveitis, Retinal Vasculitis and Autoimmune Retinopathy

Leo Meller, BS, Medical Student, First-Year, UC San Diego School of Medicine, La Jolla, CA

Andrew Lin, MD, Ophthalmology, PGY2, UC San Diego School of Medicine, La Jolla, CA; Doran Spencer, MD, Assistant Professor, Ophthalmology, UC San Diego School of Medicine, La Jolla, CA

Learning Objectives

  1. Upon completion of this lecture, learners should be better prepared to describe the ocular manifestation of Necrobiotic xanthogranuloma (NXG), especially as it relates to uveitis, retinal vasculitis and autoimmune retinopathy.

Introduction: Necrobiotic xanthogranuloma (NXG) is a rare and chronic non-Langerhans histiocytic granulomatous disease typically affecting the orbital adnexa and rarely the eye. We report the first case of NXG associated with uveitis, retinal vasculitis, and presumed autoimmune retinopathy (AIR).

Case Presentation: A 58-year-old female with a history of monoclonal gammopathy initially presented with uveitis of the right eye and was treated with topical steroids. She began to experience bilateral vision loss and eye pain, with progressive dyschromatopsia and the development of a left afferent pupillary defect. Workup revealed biopsy-proven NXG presenting as a left orbital mass. Electroretinogram showed diffuse cone-rod dysfunction with an electronegative B-wave. Fluorescein angiography revealed diffuse retinal vasculitis.

Final Diagnosis: Necrobiotic xanthogranuloma (NXG)

Management: Retinal Vasculitis was treated with rituximab. Intravenous immunoglobulin was eventually initiated due to concerns for the development of AIR. She was also referred to radiation oncology for radiotherapy for potential optic nerve compression. The patient is receiving close follow-up.

References and Resources

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