Necrobiotic xanthogranuloma (NXG) is a rare non-X histiocytosis with conspicuous lesions on the periorbital skin.
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.
Treatment with melphalan and prednisone resulted in great improvement of cutaneous lesions and paraproteinemia remission.
This case report details how melphalan and prednisone can be administered in the successful treatment of necrobiotic xanthogranuloma with lambda paraproteinemia.