Successful treatment of necrobiotic xanthogranuloma with intravenous immunoglobulin.

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.