Most cases of necrobiotic xanthogranuloma (NXG) occur in association with a monoclonal gammopathy such as MGUS. However, this study explores a much rarer possibility: that some patients may develop NXG because of an underlying genetic mutation rather than a plasma cell disorder.

This large systematic review of systemic treatments for necrobiotic xanthogranuloma (NXG) analyzed 175 patients across 79 published studies to identify which therapies have been most effective. The review found that intravenous immunoglobulin (IVIG) had the highest response rate, followed by corticosteroids and corticosteroid-based combination therapies.

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.