Necrobiotic xanthogranuloma (NXG) is an indolent non-Langerhans cell histiocytosis characterized by yellow xanthomatous plaques that tend to ulcerate. Necrobiotic xanthogranulomas have a predilection for the bilateral periorbital region and often present with consequential ophthalmic findings. Histopathology usually reveals a distinctive pattern of histiocytic xanthogranuloma with hyaline necrobiosis. Necrobiotic xanthogranuloma has been documented to have a close association with paraproteinemia. We report the case of a 76-year-old man with periorbital NXG without development of a monoclonal gammopathy. Clinically, the patient presented with dry eyes and substantial periorbital edema with multiple yellow indurated plaques. He developed the condition 30 years prior to presentation at which time it was initially diagnosed as xanthelasma. He underwent surgical excision of the lesions 10 years prior to the current presentation and biopsy results revealed a diagnosis of NXG. The periorbital lesions recurred several years prior to presentation, prompting annual computed tomography scans to rule out ocular invasion. Periorbital edema and plaques improved during a 6-month regimen of acitretin but returned to baseline just months after discontinuation.
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