MacDonald D M, Germain D, Perrot H
Br J Dermatol. 1981 Jan;104(1):7-17. doi: 10.1111/j.1365-2133.1981.tb01705.x.
The light, polarization and electron microscopic changes in the liver of four cases of erythropoietic protoporphyria (EPP) are described. In one of these cases liver involvement resulted in death, whereas in the other three there were no symptoms of referable to hepatic disease. In two cases hepatic pigment was readily visible on light and polarization microscopy and corresponded to characteristic crystalline aggregates seen ultrastructurally. Pronounced micronodular cirrhosis and cholestasis were present in the fatal case, while in the other only mild periportal fibrosis and mononuclear inflammatory cell infiltrate occurred. The third and fourth cases showed no evidence of hepatic involvement on light microscopy. Electron microscopy, however, revealed characteristic crystal-containing vacuoles indicative of protoporphyrin deposition. The need for early detection of the hepatic lesion is emphasized.
本文描述了4例红细胞生成性原卟啉病（EPP）患者肝脏的光镜、偏振光镜及电镜变化。其中1例肝脏受累导致死亡，而另外3例无肝病相关症状。2例肝脏色素在光镜和偏振光镜下易于观察到，超微结构显示为特征性的结晶聚集体。致命病例中出现明显的小结节性肝硬化和胆汁淤积，而另外3例仅出现轻度汇管区周围纤维化和单核炎性细胞浸润。第3例和第4例光镜下无肝脏受累证据。然而，电镜显示有特征性的含晶体空泡，提示原卟啉沉积。强调了早期检测肝脏病变的必要性。
