Siersema P D, Rademakers L H, Cleton M I, ten Kate F J, de Bruijn W C, Marx J J, Wilson J H
Department of Internal Medicine II, University Hospital Rotterdam-Dijkzigt, The Netherlands.
J Hepatol. 1995 Sep;23(3):259-67.
Porphyria cutanea tarda is a disorder of porphyrin metabolism, of which familial and sporadic forms have been described. Factors such as iron seem necessary for porphyria cutanea tarda to become clinically manifest. To study the relationship between iron and uroporphyrins in hepatocytes of patients with porphyria cutanea tarda, a morphological and morphometrical study was performed in 13 liver biopsies of patients with porphyria cutanea tarda (eight with sporadic porphyria cutanea tarda and five with familial porphyria cutanea tarda). In addition, possible differences in clinical and biochemical features and in histopathological findings between patients with sporadic porphyria cutanea tarda and familial porphyria cutanea tarda were investigated. Familial porphyria cutanea tarda patients presented at a younger age than sporadic porphyria cutanea tarda patients (42.4 +/- 5.3 vs. 57.3 +/- 8.6 years). Biochemical features were not different between sporadic porphyria cutanea tarda and familial porphyria cutanea tarda patients. Uroporphyrin crystals and a variable degree of liver siderosis were detected in the biopsies of all 13 patients. Uroporphyrin crystals were often found close to ferritin-like iron deposits. The morphometrical analysis showed that an increased mean area fraction of ferritin iron was associated with an increased mean area fraction of uroporphyrin crystals in hepatocytes of sporadic porphyria cutanea tarda and familial porphyria cutanea tarda patients. Moreover, the amount of uroporphyrin crystals was significantly higher in livers of familial porphyria cutanea tarda than sporadic porphyria cutanea tarda patients. These findings are consistent with the hypothesis that uroporphyria is precipitated by an iron-dependent process.(ABSTRACT TRUNCATED AT 250 WORDS)
迟发性皮肤卟啉病是一种卟啉代谢紊乱疾病，已描述有家族性和散发性两种类型。铁等因素似乎是迟发性皮肤卟啉病临床表现的必要条件。为研究迟发性皮肤卟啉病患者肝细胞中铁与尿卟啉之间的关系，对13例迟发性皮肤卟啉病患者（8例散发性迟发性皮肤卟啉病患者和5例家族性迟发性皮肤卟啉病患者）的肝脏活检组织进行了形态学和形态计量学研究。此外，还调查了散发性迟发性皮肤卟啉病患者与家族性迟发性皮肤卟啉病患者在临床和生化特征以及组织病理学结果方面的可能差异。家族性迟发性皮肤卟啉病患者发病年龄比散发性迟发性皮肤卟啉病患者小（42.4±5.3岁对57.3±8.6岁）。散发性迟发性皮肤卟啉病患者与家族性迟发性皮肤卟啉病患者的生化特征无差异。在所有13例患者的活检组织中均检测到尿卟啉晶体和不同程度的肝脏铁沉积。尿卟啉晶体常靠近铁蛋白样铁沉积物。形态计量学分析表明，散发性迟发性皮肤卟啉病和家族性迟发性皮肤卟啉病患者肝细胞中铁蛋白铁平均面积分数增加与尿卟啉晶体平均面积分数增加相关。此外，家族性迟发性皮肤卟啉病患者肝脏中的尿卟啉晶体数量明显高于散发性迟发性皮肤卟啉病患者。这些发现与尿卟啉病由铁依赖性过程引发的假说一致。（摘要截短于250字）
