Refractory Periorbital Necrobiotic Xanthogranuloma Treated With Plasma Cell-Directed Therapy

This case report describes a 57-year-old woman with necrobiotic xanthogranuloma (NXG) involving the periorbital region, skin, and liver. Her disease was unresponsive to multiple prior treatments, including IVIG, prednisone, rituximab, and orbital radiation. She was subsequently treated with the plasma cell-directed combination of daratumumab, lenalidomide, and dexamethasone (DRd). After 10 months of treatment, her M-protein (M-spike) decreased, her skin lesions improved, and repeat MRI showed resolution of the periorbital lesions. This case adds to the growing body of evidence suggesting that, in selected patients with NXG associated with a monoclonal gammopathy, targeting the underlying plasma cell disorder may be an effective treatment strategy.

Trando A, et al. “Refractory Periorbital Necrobiotic Xanthogranuloma Successfully Treated With Plasma Cell-Directed Therapy.” eJHaem. 2026.