Periorbital necrobiotic xanthogranuloma resolved with three years of systemic lenalidomide treatment.

Purpose: To describe a case report of the successful management of necrobiotic xanthogranuloma (NXG), a rare periorbital disease. Observations: A 61-year-old patient presented with bilateral upper and lower lid lesions which were initially misdiagnosed as xanthelasmas and later confirmed to be NXG. Further investigation also uncovered a diagnosis of multiple myeloma. The patient was started on a lenalidomide treatment for his multiple myeloma, specifically chosen by a coalition of Hematology/Oncology, Dermatology, Internal Medicine and Oculoplastics, to simultaneously treat his NXG. His periocular lesions were monitored for response without additional intervention. It was initially difficult to determine if the patient's periocular involvement was improving due to conflicting concurrent ulceration and decreased height of the lesions. At 18 months, improvement became apparent. After three years of treatment, the patient's periocular lesions had clinically disappeared. Follow-up with the patient confirmed that there had been no return of either the NXG or multiple myeloma three years after treatment cessation. Conclusions and Importance: This case contributes to the existing literature by documenting complete resolution of NXG after three years of lenalidomide treatment, which is a much longer treatment duration than typically described in the literature. These observations alert treating physicians that a longer treatment interval may be needed to address NXG.