Chang Chao-Fu, Lin Chih-Ching, Chen Jinn-Yang, Yang An-Hang, Shiao Ming-Shi, Kao Jau-Tsuen, Yang Wu-Chang
Division of Nephrology, Department of Medicine, Taipei Veterans General Hospital, Taiwan, China.
Am J Kidney Dis. 2003 Sep;42(3):E18-23. doi: 10.1016/s0272-6386(03)00798-4.
Lipoprotein glomerulopathy (LPG) is a rare disease, characterized by a special histology, including dilated glomerular capillaries filled with pale-stained and meshlike lipoprotein thrombi. It always presents with proteinuria or nephrotic syndrome. Although hyperlipidemia is not always seen, most patients have type III hyperlipoproteinemia with apolipoprotein (apo) E2/3 phenotyping. Although the clinical feature of LPG is rarely described, LPG associated with other glomerulopathy, including IgA nephropathy, membranous nephropathy, and lupus nephritis, has been documented. Until now, there have been no reports of psoriasis vulgaris associated with LPG. The authors present 2 cases of LPG with apo E3/3 genotyping associated with psoriasis vulgaris. The first patient was a 65-year-old woman who presented with nephrotic syndrome with daily urinary protein loss of 9.05 g and itchy erythematous scaly plaques on her trunk and lower limbs for 1 year. The renal biopsy results showed LPG, and the skin biopsy results showed psoriasis. The second patient was a 50-year-old man with history of psoriasis over his trunk and 4 limbs for 30 years. He also presented with nephrotic syndrome with daily urinary protein loss of 7.55 g. The renal biopsy results also showed LPG. The genotype of apo E showed E3/3, and lipoprotein electrophoresis showed a type III hyperlipoproteinemia-like pattern in both cases. The authors suggest that presence of apo E3/3 genotype cannot rule out the diagnosis of type III hyperlipoproteinemia and LPG. Besides, LPG should be included in the differential diagnosis of psoriatic patients with nephrotic syndrome, especially in Asian patients who show poor response to traditional therapy. Renal biopsy should be performed to make the definitive diagnosis.
脂蛋白肾小球病（LPG）是一种罕见疾病，其特征为特殊的组织学表现，包括肾小球毛细血管扩张，内充满淡染的网状脂蛋白血栓。该病常表现为蛋白尿或肾病综合征。虽然并非总是可见高脂血症，但大多数患者为Ⅲ型高脂蛋白血症，载脂蛋白（apo）表型为E2/3。尽管LPG的临床特征鲜有描述，但已记录到LPG与其他肾小球病相关，包括IgA肾病、膜性肾病和狼疮性肾炎。迄今为止，尚无寻常型银屑病与LPG相关的报道。作者报告了2例apo E3/3基因分型的LPG合并寻常型银屑病的病例。首例患者为一名65岁女性，表现为肾病综合征，每日尿蛋白丢失9.05g，躯干和下肢出现瘙痒性红斑鳞屑斑块1年。肾活检结果显示为LPG，皮肤活检结果显示为银屑病。第二例患者为一名50岁男性，躯干和四肢患银屑病30年。他也表现为肾病综合征，每日尿蛋白丢失7.55g。肾活检结果也显示为LPG。apo E基因型显示为E3/3，两例患者的脂蛋白电泳均显示出类似Ⅲ型高脂蛋白血症的模式。作者提示，apo E3/3基因型的存在不能排除Ⅲ型高脂蛋白血症和LPG的诊断。此外，对于肾病综合征的银屑病患者，尤其是对传统治疗反应不佳的亚洲患者，鉴别诊断时应考虑LPG。应进行肾活检以明确诊断。
