Department of Renal Medicine, Concord Repatriation General Hospital, Hospital Rd, Concord, NSW, Australia.
Department of Anatomical Pathology, Concord Repatriation General Hospital, Hospital Rd, Concord, NSW, Australia.
J Nephrol. 2022 Jul;35(6):1753-1757. doi: 10.1007/s40620-021-01186-9. Epub 2021 Nov 5.
Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans cell histiocytosis with characteristic cutaneous features and rare visceral involvement. More than 80% of individuals with this disease have a detectable paraprotein but the precise pathogenesis remains obscure. A 68-year-old man with known cutaneous necrobiotic xanthogranuloma presented with acute kidney injury and imaging suggestive of bilateral perinephric infiltration. Renal biopsy showed a prominent histiocytic infiltration of renal capsule and cortex with necrobiosis and characteristic 'Touton-type' giant cells suggestive of necrobiotic xanthogranuloma involvement. Kidney function returned to normal and cutaneous lesions improved with a combination of corticosteroid, chlorambucil and rituximab. This case represents only the second reported incidence of kidney involvement by necrobiotic xanthogranuloma and the first with acute kidney injury and pre-mortem histopathology. This report adds to a small body of literature on the diagnosis and management of visceral involvement by this rare disease.
坏死性黄色肉芽肿（NXG）是一种罕见的非朗格汉斯细胞组织细胞增生症，具有特征性的皮肤表现和罕见的内脏受累。超过 80%的该病患者可检测到单克隆丙种球蛋白，但确切的发病机制仍不清楚。一名 68 岁男性患有已知的皮肤坏死性黄色肉芽肿，出现急性肾损伤和影像学提示双侧肾周浸润。肾活检显示肾包膜和皮质有明显的组织细胞浸润，伴有坏死和特征性的“Touton 样”巨细胞，提示坏死性黄色肉芽肿受累。肾功能恢复正常，皮肤病变在皮质类固醇、苯丁酸氮芥和利妥昔单抗联合治疗后得到改善。本例是坏死性黄色肉芽肿肾脏受累的第二例报道，也是首例伴有急性肾损伤和生前组织病理学的报道。本报告增加了关于这种罕见疾病内脏受累的诊断和治疗的少量文献。
