Mustajoki P, Koskelo P
Acta Med Scand. 1976;200(3):171-8. doi: 10.1111/j.0954-6820.1976.tb08216.x.
The occurrence of hepatic porphyrias--acute intermittent porphyria (AIP) and variegate porphyria (VP)--in Finland has been studied. During a period of 9 years 107 patients with AIP and 45 patients with VP were found. The prevalence of hereditary hepatic porphyrias was calculated to be 3.4 per 100 000 inhabitants. The patients belonged to 42 different families. Eighty-nine patients (59%) had had acute attacks, whereas 63 were symptomless latent cases. Precipitating factors, symptoms and excretion of porphyrins and their precursors did not significantly differ from what has been reported earlier from other parts of the world. A slight fragility of the skin on the back of the hands was noted in some 50% of VP patients. Abnormal sensitivity to sunlight could not be seen in a single case. However, about 50% of patients with VP showed an abnormal reaction when irradiated with artificial ultraviolet light. The difference in the skin symptoms in South African and Finnish VP patients is discussed.
对芬兰肝性卟啉病——急性间歇性卟啉病（AIP）和混合型卟啉病（VP）的发病情况进行了研究。在9年的时间里，共发现107例AIP患者和45例VP患者。遗传性肝性卟啉病的患病率经计算为每10万居民中有3.4例。这些患者分属42个不同的家庭。89例患者（59%）曾有过急性发作，而63例为无症状的潜伏病例。诱发因素、症状以及卟啉及其前体的排泄情况与世界其他地区先前报道的情况相比无显著差异。约50%的VP患者手部背部皮肤略显脆弱。未发现有一例对阳光异常敏感。然而，约50%的VP患者在接受人工紫外线照射时会出现异常反应。文中讨论了南非和芬兰VP患者皮肤症状的差异。
