Necrobiotic xanthogranuloma: A case report of successful treatment with intravenous immunoglobulins

Abstract Necrobiotic xanthogranuloma (NXG) is a rare non‐Langerhans cell histiocytosis associated with paraproteinemia. Skin lesions of NXG are difficult to treat and various therapies have been evaluated with inconsistent results. A therapeutic standard has not been established. We report on a patient severely affected by mutilating skin and ocular lesions as well as lung infiltrates of necrobiotic xanthogranuloma. After failure of several previous therapies including dapsone and adalimumab, lesions significantly improved during therapy with intravenous immunoglobulins (IVIg). IVIg are an effective treatment option to evaluate in NXG.