慢性肾衰血液透析(慢透)患者约1％出现与迟发性皮肤卟啉病不能鉴别的皮损。一般来说并无卟啉代谢异常的证据,但曾报道过三例慢透患者发生真的迟发性皮肤卟啉病。原发性酶缺陷为肝内尿卟啉原脱羧酶活性降低。本文报告一例为此种酶的遗传性缺陷患者,于慢透时发病。患童16岁,因慢性肾小球肾炎,于1975年8月起 About 1% of patients with chronic renal failure who have hemodialysis (slowly penetrating) develop lesions that can not be distinguished from those of late-onset cutaneous porphyria. In general, there is no evidence of abnormalities in porphyrin metabolism, but it has been reported that three cases of truly late-onset skin porphyria occur slowly. Primary enzyme deficiency is the reduction of intrahepatic uroporphyrinogen decarboxylase activity. This article reports a case of hereditary defects in this enzyme in patients with the onset of slow through. 16-year-old child suffering from chronic glomerulonephritis, in August 1975 onwards