Background: Necrobiotic xanthogranuloma (NXG) is a rare chronic disorder characterized by firm yellow to red-orange nodules and plaques affecting the face, abdomen, and extremities with the potential for systemic involvement. NXG has a close association with monoclonal gammopathies, and there is a predilection for the development of multiple myeloma. Treatment options are varied due to inconsistent results seen with the use of corticosteroids, immunomodulators, chemotherapeutic agents, and antibiotics. Objective: We describe a patient with smoldering multiple myeloma associated with progressive NXG successfully treated with high-dose intravenous immunoglobulin (IVIG). Conclusion: Our case adds to the single previous report of two cases of NXG with significant improvement from treatment with IVIG and confirms the efficacy of this treatment modality.
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