[Does paraneoplastic porphyria cutanea tarda exist?].

E Köstler
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Abstract

Our investigations in 134 patients showed corresponding to literature porphyria cutanea tarda (PCT) diagnosed by biochemical methods not to be a paraneoplastic dermatosis (but one possible exception acquainted). Relations between PCT and extrahepatic non-porphyrin producing tumours are improbable. Nevertheless but extremely seldom an irregular urinary porphyrin excretion associated with cutaneous changes of hepatic porphyria should lead to the presumption of a porphyrin producing hepatoma. PCT lasting for decades apparently presents a higher frequency of hepatocellular carcinoma in patients suffering from liver cirrhosis than in cirrhotics without PCT. It is supposed that this possible progredience of liver disease in PCT into hepatocellular carcinoma may be prevented by chloroquine phosphate therapy.

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是否存在迟发性副肿瘤卟啉症?
我们对134例患者的调查显示,通过生化方法诊断的迟发性皮肤卟啉症(PCT)不是一种副瘤性皮肤病(但有一个可能的例外)。PCT与肝外非卟啉产生肿瘤之间的关系不大可能。然而,极少有与肝性卟啉的皮肤改变相关的不规则尿卟啉排泄会导致卟啉产生性肝癌的推定。经过数十年PCT治疗的肝硬化患者发生肝细胞癌的频率明显高于未进行PCT治疗的肝硬化患者,我们推测,磷酸氯喹治疗可以预防PCT患者肝病发展为肝细胞癌的可能性。
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