伴IgG—κ型副球蛋白血症和眼睛受累的渐进坏死性黄色肉芽肿

Necrobiotic xanthogranuloma with immunoglobulin G kappa paraproteinemia and eye involvement

患者男，74岁。躯干黄红色斑块1年余。体检示躯干散在20余个黄红色结节斑块，直径0．5～15em，多数不规则形，境界清楚。右下腹斑块破溃萎缩，较多结痂。血浆蛋白电泳示免疫球蛋白G18．3g／L，κ轻链20．70g／L。组织病理示真皮和皮下组织交替形成大片渐进性坏死和肉芽肿。肉芽肿含大量Touton巨细胞、异物巨细胞、泡沫细胞等。在渐进性坏死区可见多数胆固醇裂隙，部分区域可见淋巴滤泡形成。患者双眼不同程度受累。诊断为伴IgG—κ型副球蛋白血症和眼睛受累的渐进坏死性黄色肉芽肿。

A 74-year-old man presented with yellowish red nodules and plaques on the trunk for more than one year. Physical examination showed more than 20 scattered, irregularly shaped, well-demarcated, yellowish red nodules and plaques measuring 0.5 - 15 cm in diameter on the trunk. The plaques on the right lower abdomen showed central ulceration and atrophy with scar formation. Plasma protein electrophoresis revealed that the levels of immnnoglobulin G （IgG） and kappa light chain were 18.3 g/L and 20.70 g/L respectively. Histopathologically, large necrobiotic areas and granuloma were observed alternately in the dermis and subcutis. There were numerous Touton giant cells, foreign body giant cells and fomny histioeytes in the granuloma, and many cholesterol clefts were found in the center of necrobiotie areas with the formation of lymphoid follicles in some regions. Both eyes of the patient were involved to different degrees. He was diagnosed as necrobiotie xanthogranuloma with IgG kappa paraproteinemia and eye involvement.