Blepharoxanthogranuloma: A Rare Ocular Manifestation Of Xanthogranuloma Necrobioticum

The purpose of this study is to better understand the clinical course and management approaches for blepharoxanthogranuloma, a rare ocular manifestation of necrotizing xanthogranuloma that is frequently often associated with systemic diseases such as monoclonal gammopathy. This will emphasize the value of multidisciplinary cooperation and ongoing observation.

: Blepharoxanthogranuloma, Necrotizing xanthogranuloma, Monoclonal gammopathy, Multidisciplinary approach, Continuous monitoring

This case study follows a 56-year-old woman whose skin lesions initially appeared in 2013 and whose diagnosis of necrotizing xanthogranuloma was confirmed by biopsy in 2015. Hematological analysis in 2022 found bone marrow plasma cell infiltration; the level of immunoglobulin G (IgG) was found to be 47.5 g/L. Systemic treatment with Decortin and Ciprilon was initiated, followed by lenalidomide and dexamethasone. In 2022, the paraorbital lesions improved, but the infraorbital suppuration still persisted. The ulcer on the left eye was fully epithelialized by 2022, however there was a 2-3 mm purulent patch in the right infraorbital area. The monitoring of treatment response underscored the need for a multidisciplinary approach.

The patient underwent monitoring and regular follow ups for a duration of four years, having regulary treatment with topical, intralesion and systemic corticosterioids. Our ophthalmological examination in 2024 revealed posterior capsule cataracts and paraocular scar lesions. Both eyes' corrected visual acuity was 20/63 BCVA, necessitating cataract surgery. Multiple periocular scarring lesions were seen on both sides during an eye checkup in 2024, showing that the illness process is persistent. Even while some lesions successfully epithelialized and showed signs of partial healing, further monitoring indicated that therapy was still necessary due to persisting problems.

This case contributes to the limited literature on blepharoxanthogranuloma, highlighting the complexities of its management and shows the importance of long-term monitoring to prevent complications. Blepharoxanthogranuloma management requires a multidisciplinary approach involving dermatologists, hematologists, and ophthalmologists. Continuous monitoring is crucial for optimizing patient outcomes and enhancing our understanding of this rare ocular condition.