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. Lenalidomide-containing regimens also showed promising results in many patients. Based on the available evidence, the authors conclude that IVIG and corticosteroids should be considered first-line treatments for NXG, while acknowledging that larger prospective studies are needed due to the rarity of the disease.
Notably, the review found that approximately 65% of reported NXG patients also had an associated monoclonal gammopathy or hematologic malignancy, reinforcing the importance of evaluation by a hematologist.
Miguel D, Lukács J, Elsner P. “A Systematic Review of Treatments for Necrobiotic Xanthogranuloma.” Orphanet Journal of Rare Diseases. 2022.
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