Freesemann A, Frank M, Sieg I, Doss M O
Division of Clinical Biochemistry, Philipps University, Marburg, Germany.
Skin Pharmacol. 1995;8(3):156-61. doi: 10.1159/000211340.
Porphyria cutanea tarda (PCT) is characterized by cutaneous symptoms in association with hepatic accumulation and urinary excretion of mainly uro- and heptacarboxyporphyrins. 24 PCT patients excreting urinary porphyrins between 1.9 and 5.8 mumol/24 h (normal < 0.2) were treated with chloroquine at a dose of 125 mg twice a week. During the first phase of therapy urinary porphyrin excretion transiently increased 1.1- to 2.9-fold indicating a phase of mobilization. A slight initial elevation was also found regarding the activities of serum aminotransferases reflecting the hepatotoxic effect of chloroquine. The clinical symptoms disappeared after a period ranging from 2 to 6 months, and after an average of 12 months the porphyrin excretion in all patients had returned to nearly normal values.
迟发性皮肤卟啉症(PCT)的特征是皮肤症状与肝脏中主要是尿卟啉和七羧基卟啉的蓄积及尿排泄有关。24例尿卟啉排泄量在1.9至5.8μmol/24小时之间(正常<0.2)的PCT患者,接受了每周两次、每次125mg氯喹的治疗。在治疗的第一阶段,尿卟啉排泄量短暂增加了1.1至2.9倍,表明有一个动员阶段。血清转氨酶活性也有轻微的初始升高,这反映了氯喹的肝毒性作用。临床症状在2至6个月后消失,平均12个月后,所有患者的卟啉排泄量已恢复到几乎正常的值。