Necrobiotic xanthogranuloma of the extremities with paraproteinemia and without periorbital involvement at presentation.

We report a rare case of necrobiotic xanthogranuloma (NXG) of the extremities with paraproteinemia and without periorbital involvement at presentation in a 58-year-old white woman. A combination of oral cyclophosphamide and oral dexamethasone was attempted, but the patient then developed a left intraorbital lesion. Treatment was not successful in that the gammopathy did not improve and the patient continued to develop more lesions.