KOVAL A, DANBY C W, PETERMANN H
Can Med Assoc J. 1965 Sep 4;93(10):537-40.
CURRENTLY, THE PORPHYRIAS ARE CLASSIFIED IN FOUR MAIN GROUPS: congenital porphyria, acute intermittent porphyria, porphyria cutanea tarda hereditaria, and porphyria cutanea tarda symptomatica. The acquired form of porphyria (porphyria cutanea tarda symptomatica) occurs in older males and is nearly always associated with chronic alcoholism and hepatic cirrhosis. The main clinical changes are dermatological, with excessive skin fragility and photosensitivity resulting in erosions and bullae. Biochemically, high levels of uroporphyrin are found in the urine and stools. Treatment to date has been symptomatic and usually unsuccessful.A case of porphyria cutanea tarda symptomatica is presented showing dramatic improvement of both the skin lesions and porphyrin levels in urine and blood following repeated phlebotomy.Possible mechanisms of action of phlebotomy on porphyria cutanea tarda symptomatica are discussed.
目前,卟啉症主要分为四大类:先天性卟啉症、急性间歇性卟啉症、遗传性迟发性皮肤卟啉症和症状性迟发性皮肤卟啉症。获得性卟啉症(症状性迟发性皮肤卟啉症)多见于老年男性,几乎总是与慢性酒精中毒和肝硬化有关。主要临床变化为皮肤方面的,皮肤过度脆弱和光敏性导致糜烂和大疱。在生化方面,尿液和粪便中发现高水平的尿卟啉。迄今为止的治疗一直是对症治疗,通常并不成功。本文报告了一例症状性迟发性皮肤卟啉症病例,该病例显示在反复放血后,皮肤病变以及尿液和血液中的卟啉水平均有显著改善。文中讨论了放血治疗症状性迟发性皮肤卟啉症的可能作用机制。