Objective To explore the characteristics of patients with post-infectious glomerulonephritis in epidermolysis bullosa (EB),and improve the understanding of the disease.Methods The clinical data of one case of post-infectious glomerulonephritis in EB in our hospital were retrospectively analyzed,and the related literatures were reviewed to examine the clinical features and prognosis of this condition.Results A 52-year-old man exhibited EB at birth,abnormal urine test was found due to a cough two months ago,the renal function and complement were normal,and ASO was not high.The skin biopsy showed squamous epithelium edema,looseness and blister formation,and immunofluorescence was all negative.Renal biopsy showed glomerular segmental mild mesangial proliferative lesion.The immunofluorescence C3 + distributed diffusely,and IgA + segments were granular in the mesangial region.An isolated cluster of electron dense deposits was observed in the mesangial area under the electronic microscopy.The final diagnosis was post-infectious glomerulonephritis in congenital bullous epidermolysis.The patient was in stable condition with small dose of glucocorticoids combined with tripterygium glycoside tablets.Conclusions The occurrence of renal damage in patients with congenital epidermolysis is extremely rare in clinic.As a clinician,it is necessary to strengthen the examination of urine and kidney function in patients with EB,and renal biopsy is necessary to provide the basis for treatment.%目的 分析先天性大疱表皮松解症(EB)合并感染后肾小球肾炎的发病特点,提高临床医生对该疾病的认识.方法 对南京总医院肾脏病研究所1例EB合并感染后肾小球肾炎患者的临床资料进行回顾性分析,并对相关文献进行复习,分析该特殊人群的发病特点,探讨可能的发病机制.结果 本例患者男性,52岁,自幼即反复出现全身皮肤水疱、大疱、出血伴结痂,间断小剂量强的松治疗效果不佳,2月余前因咳嗽就诊发现尿检异常,肾功能正常.本次入院后查抗链球菌溶血素O不高,补体正常,行皮肤活检示鳞状上皮棘层部分区域水肿松解伴水疱形成、免疫荧光全阴性,肾活检示肾小球节段轻度系膜增生性病变伴体积增大、球性废弃,免疫荧光C3+弥漫、IgA+节段呈颗粒状分布于系膜区,电镜见1处系膜旁区见孤立的团块状电子致密物沉积,最终诊断为EB合并感染后肾小球肾炎,经小剂量激素联合雷公藤多苷片及大黄制剂等治疗,患者病情稳定.结论 EB患者发生肾损害在临床极为罕见,作为临床医生应加强对EB患者相关尿液及肾功能检查,必要时行肾活检明确诊断为治疗提供依据.
展开▼
