Background: Necrobiotic xanthogranuloma (NXG) is a rare non-X histiocytosis with conspicuous lesions on the periorbital skin.
Methods: A diabetic patient presented with NXG and a previous diagnosis of necrobiosis lipoidica on the legs over a period of almost 2 years before the development of the typical lesions of NXG on the periorbital regions, back, thighs and legs. The patient was found also to have developed lambda paraproteinemia.
Results: Treatment with melphalan and prednisone resulted in great improvement of cutaneous lesions and paraproteinemia remission.
Conclusion: This case report details how melphalan and prednisone can be administered in the successful treatment of necrobiotic xanthogranuloma with lambda paraproteinemia.