Purpose

Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans histiocytosis associated with monoclonal gammopathy. We highlight the role of oculoplastic surgeons in early recognition and referral through a case illustrating delayed diagnosis and systemic progression.

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Methods

We report a case of NXG with multidisciplinary assessment of clinical, imaging, and laboratory findings.

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Results

An 82-year-old female presented with bilateral eyelid lesions; excision and histopathology confirmed NXG.
She had a history of nonspecific systemic symptoms and weight loss, with multiple evaluations, including haematological assessment, failing to establish the true underlying cause.
Despite further investigations, the condition remained unrecognized for a prolonged period. She subsequently developed progressive multisystem involvement, including cutaneous, orbital, pulmonary, and hepatic manifestations, initially suggestive of disseminated malignancy.
Laboratory evaluation showed anaemia, leukopenia, elevated ESR, elevated total protein, and impaired renal function. Serum protein electrophoresis revealed a prominent gamma fraction with a high M-component. Ultimately, immunofixation and bone marrow biopsy established IgG kappa multiple myeloma.
Following daratumumab-based therapy (D-VRd), rapid clinical and radiological remission was achieved.

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Conclusion

NXG may represent a marker of monoclonal gammopathy and warrants systemic evaluation to avoid diagnostic delay and progression to a potentially life-threatening disease.
Histopathological diagnosis of NXG should prompt immediate haematological referral, including serum protein electrophoresis and immunofixation.

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Additional Authors

First name	Last name	Base Hospital / Institution
Olga	Lukáts	Semmelweis University, Department of Ophthalmology
Márton	Magyar	Semmelweis University, Department of Ophthalmology
Nóra	Szentmáry	Semmelweis University, Department of Ophthalmology

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