Necrobiotic xanthogranuloma of the eyelids with paraproteinemia

Abstract 

Necrobiotic xantogranuloma (NXG) with paraproteinemia is characterized by multiple nodules or plaques that involve the periorbital area. The lesions frequently become inflamed leading to superficial ulceration; multiple myeloma is present in some patients. A case of NXG with IgG monoclonal gammopathy is reported; the ocular symptoms were clinically pre-eminent. Histologically the granulomas are characterized by collagen necrobiosis, many foamy histiocytes, Touton giant cells, bizarre foreign body cells, and were positive for light chain kappa. In this patient, chemotherapy did not have a favorable response, therefore the lesions were excised and the resulting defects were covered with split skin grafts. The patient died seven months after surgical treatment, this was the result of complications of myeloma.