Abstract: Necrobiotic xanthogranuloma is easy to be misdiagnosed as progressive lipoid necrosis, xanthelasma palpebrarum, and annular granuloma. In this case, yellow-red plaques were scattered on the eyelid, periorbital skin, neck and waist, and histopathology was consistent with necrobiotic xanthogranuloma. Serum immunofixation electrophoresis showed protein of monoclonal IgG and Kappa light chain. There is no standard treatment and the reported treatment methods included systemic use of glucocorticoids, high-dose injection of immunoglobulin, methotrexate, thalidomide, rituximab, interferon-α, TNF-α inhibitors and antimalarial drugs. This patient was treated with methylprednisolone, thalidomide, and total glucosides of paeony, the skin lesions were significantly improved after 7 months, and the patient is under follow-up.

Key words: necrobiotic xanthogranuloma, paraproteinemia, treatment