Adnexal necrobiotic xanthogranuloma presenting as a “kissing” eyelid lesion

Necrobiotic xanthogranuloma (NBX; also termed NXG) is a rare non‑Langerhans cell histiocytosis within the adult orbital xanthogranulomatous disease spectrum. It typically presents with uni‑ or bilateral periocular yellow‑brown infiltrates and can mimic xanthelasma and other inflammatory lesions, with possible systemic associations including paraproteinemia and hematologic malignancy. A 50‑year‑old man presented with an uncommon phenotype limited to the ipsilateral upper and lower eyelids, forming a "kissing" lesion across the lid margin. Three prior debulking procedures performed elsewhere without histopathology resulted in recurrence. A shave biopsy demonstrated foamy histiocytes, multinucleated giant cells, lymphocytes, and necrobiosis, consistent with NBX. Systemic evaluation revealed no paraproteinemia, amyloidosis, IgG4‑related disease, immunodeficiency, or hematologic malignancy. Five intra‑/perilesional corticosteroid injections (triamcinolone; upper lid 0.3 mL [12 mg], lower lid 0.1 mL [4 mg]; 3-9‑month intervals) produced a limited response. Lesions remain stable 2.5 years after the last injection. NBX can rarely present as an isolated "kissing" eyelid lesion. Early histopathologic confirmation helps avoid repeated procedures and triggers appropriate systemic work‑up and longitudinal surveillance.

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Publication Details

Journal
Orbit
Published
2026-04-21
DOI
https://doi.org/10.1080/01676830.2026.2661253
Primary Topic
Histiocytic Disorders and Treatments
Type
article
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article

Adnexal necrobiotic xanthogranuloma presenting as a “kissing” eyelid lesion

D. Scheers, D. Paridaens, J. A. M. van Laar, M. A. Suadi et al.
Orbit
Histiocytic Disorders and Treatments
article

Adnexal necrobiotic xanthogranuloma presenting as a “kissing” eyelid lesion

D. Scheers, D. Paridaens, J. A. M. van Laar, M. A. Suadi, R. M. Verdijk, S. E. Detiger
article en

Abstract

Necrobiotic xanthogranuloma (NBX; also termed NXG) is a rare non‑Langerhans cell histiocytosis within the adult orbital xanthogranulomatous disease spectrum. It typically presents with uni‑ or bilateral periocular yellow‑brown infiltrates and can mimic xanthelasma and other inflammatory lesions, with possible systemic associations including paraproteinemia and hematologic malignancy. A 50‑year‑old man presented with an uncommon phenotype limited to the ipsilateral upper and lower eyelids, forming a "kissing" lesion across the lid margin. Three prior debulking procedures performed elsewhere without histopathology resulted in recurrence. A shave biopsy demonstrated foamy histiocytes, multinucleated giant cells, lymphocytes, and necrobiosis, consistent with NBX. Systemic evaluation revealed no paraproteinemia, amyloidosis, IgG4‑related disease, immunodeficiency, or hematologic malignancy. Five intra‑/perilesional corticosteroid injections (triamcinolone; upper lid 0.3 mL [12 mg], lower lid 0.1 mL [4 mg]; 3-9‑month intervals) produced a limited response. Lesions remain stable 2.5 years after the last injection. NBX can rarely present as an isolated "kissing" eyelid lesion. Early histopathologic confirmation helps avoid repeated procedures and triggers appropriate systemic work‑up and longitudinal surveillance.

Orbit
Erasmus MC (NL), Rotterdam Eye Hospital (NL)
Openalex Percentile: Top 6%
Histiocytic Disorders and Treatments
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