Abstract：
Necrobiotic xanthogranuloma (NXG) is a progressive non-Langerhans cell histiocytosis with a predilection for the periorbital area. NXG is most commonly associated with monoclonal gammopathy and ophthalmic complications. The authors present a 69-year-old man who was evaluated for a left upper eyelid nodule and plaques on the lower extremities, trunk, abdomen, and right upper extremity. Biopsy of the eyelid was supportive for NXG. Serum protein electrophoresis was positive for a monoclonal gammopathy, IgG light chain kappa. MRI showed preseptal involvement. The periocular nodules cleared with a high dose of prednisone; however, the other skin lesions persisted. Bone marrow biopsy showed kappa-restricted 6% plasma cells and he was treated with intravenous immunoglobulin. This case illustrates the importance of clinicopathologic correlations to render an NXG diagnosis.
摘要：
坏死生物黄色肉芽肿（NXG）是一种进行性非朗格汉斯细胞组织细胞增生症，对眶周区域有好感。NXG通常与单克隆丙种球蛋白病和眼科并发症有关。作者介绍了一名69岁的男子，他接受了左上睑结节和下肢斑块的评估，树干,腹部，和右上肢。眼睑活检支持NXG。血清蛋白电泳对单克隆丙种球蛋白病呈阳性，IgG轻链κ。MRI显示脑前受累。用大剂量泼尼松清除眼周结节；然而，其他皮肤损伤持续存在。骨髓活检显示Kappa限制性的6％浆细胞，并接受静脉注射免疫球蛋白治疗。此病例说明了临床病理相关性对NXG诊断的重要性。
