Wells M M, Golitz L E, Bender B J
Arch Dermatol. 1980 Apr;116(4):429-32.
Cholestatic jaundice and rapidly deteriorating hepatic function developed in a 19-year-old man with a lifelong history of photosensitivity. Laboratory studies revealed the characteristic increased erythrocyte and fecal protoporphyrin levels of erythropoietic protoporphyria. Progressive hepatic failure was treated by orthotopic liver transplantation six months after the first clinical indication of hepatic dysfunction. Characteristic light microscopic, fluorescence microscopic, and electron microscopic findings of erythropoietic protoporphyria were present in skin and liver. Four weeks after liver transplantation, the patient died of disseminated candidiasis. At autopsy, the donor liver had no microscopic evidence of protoporphyrin accumulation, although tissue protoporphyrin levels were mildly elevated.
一名有光敏症终生病史的19岁男性出现了胆汁淤积性黄疸和迅速恶化的肝功能。实验室检查显示了红细胞生成性原卟啉病特征性的红细胞和粪便中原卟啉水平升高。在出现肝功能障碍的首个临床迹象六个月后，通过原位肝移植治疗进行性肝衰竭。皮肤和肝脏中存在红细胞生成性原卟啉病特征性的光镜、荧光镜和电镜表现。肝移植四周后，患者死于播散性念珠菌病。尸检时，尽管组织原卟啉水平轻度升高，但供体肝脏没有原卟啉积累的镜下证据。
