Hannoud S, Senouci K, Sbai M, Benzekri L, Hassam B, Balafrej L, Ouzeddoune N, Heid E
Service de dermatologie, CHU Ibn-Sina, Rabat, Maroc.
Rev Med Interne. 2004 Apr;25(4):306-9. doi: 10.1016/j.revmed.2004.01.006.
Porphyria cutanea tarda (PCT) is a disorder of heme biosynthesis resulting from deficiency in the enzyme uroporphyrinogen decarboxylase. In the sporadic form of PCT, there are many agents that trigger the clinical manifestations.
We report a case of PCT in an hemodialysed patient with hepatitis C virus infection (HVC). He was treated with small repeated phlebotomies of 50 ml every week with photoprotection, eviction of traumatismes and inducing drugs. A clinical remission was induced after five months of treatment.
A proper diagnosis of PCT in non uremic hemodialysed patients requires fractionation of serum and fecal porphyrin changes. Management of this patients is difficult. Small repeated phlebotomies (50-100 ml) could be an interesting therapy.
迟发性皮肤卟啉症(PCT)是一种由于尿卟啉原脱羧酶缺乏导致的血红素生物合成障碍。在散发性PCT中,有许多因素可引发临床表现。
我们报告一例丙型肝炎病毒感染(HVC)的血液透析患者发生PCT的病例。对其进行每周50毫升的小剂量重复放血治疗,并采取光保护措施,避免外伤和诱发药物。治疗五个月后实现临床缓解。
对于非尿毒症血液透析患者的PCT进行准确诊断需要对血清和粪便卟啉变化进行分级。该类患者的管理较为困难。小剂量重复放血(50 - 100毫升)可能是一种有效的治疗方法。