无渐进坏死性黄色肉芽肿伴IgG κ型副球蛋白血症1例

A Case of Nonnecrobiotic Necrobiotic Xanthogranuloma with Immunoglobulin G Kappa Paraproteinemia

患者女,58岁。面颈部橘红色斑块5年。皮肤科情况:面部。尤其右眼眶周围和颈部融合性橘红色斑块、结节。皮损边界清楚,部分皮疹边缘可见红晕。血清免疫电泳示单克隆IgG k轻链。组织病理:表面萎缩变薄,真皮全层弥漫性组织细胞,泡沫细胞及多核巨细胞浸润,部分区域可见散在淋巴细胞浸润,未见明显渐进性坏死。诊断为无渐进坏死性黄色肉芽肿伴IgG κ型副球蛋白血症。

A 58-year-old woman suffered progressive red-orange nodules and plaques involving the face and neck for 5 years. Physical examination revealed indurated red-orange nodules and plaques with erythematous borders covering the face （especially the right periorbital areas ） and the neck. A monoclonal IgG kappa light chains were detected by serum protein immunoelectrophoresis. Histologic examination revealed dense infiltrates of macrophages with some foreign-body-type giant cell and foamy macrophages were accompanied by a moderate lymphocytic cells. However, evident zones of degenerated collagen and cholesterol clefts were missing. A diagnosis of nonnecrobiotic necrobiotic xanthogranuloma with immunoglobulin G kappa paraproteinemia was made.