Central laboratory, Triemli Hospital, Zurich, Switzerland,
J Inherit Metab Dis. 2010 Dec;33 Suppl 3:S465-9. doi: 10.1007/s10545-010-9234-z. Epub 2010 Nov 11.
Erythropoietic protoporphyria (EPP) is a hereditary disorder caused by deficiency of ferrochelatase, the last enzyme in the heme biosynthetic pathway. The majority of EPP patients present with a clinical symptom of painful phototoxicity. Liver damage, the most serious complication of EPP, occurs in <5% of the patients. This report describes a case of an EPP patient who complained of worsening cutaneous symptoms, nervousness, and insomnia. Laboratory tests showed highly increased protoporphyrin concentration in erythrocytes and elevated serum transaminases that are indicative of EPP-related liver damage. The subsequent finding of decreased serum thyroid-stimulating hormone (TSH) and increased free triiodothyronine (FT3) and free thyroxine (FT4) concentrations, as well antibodies against both thyroid peroxidase (TPO) and TSH receptors, led to the diagnosis of Graves' disease. The patient received 500 MBq of radioiodine (I(131)). Three months after the radioactive iodine therapy, the thyroid volume was reduced to 30% of pretherapeutic volume. Although the patient was slightly hypothyroidic, his liver enzymes returned to normal, his erythrocytic protoporphyrin concentration dropped fivefold, and his skin symptoms improved dramatically. The coexistence of Graves' disease and EPP is a statistically rare event as, besides our patient, there was one additional case reported in the literature. Although the exact mechanism whereby Graves' disease interacts with EPP is yet to be explored, we recommend testing thyroid function in EPP patients with liver complication to exclude hyperthyroidism as a potential cause.
红细胞生成性原卟啉症(EPP)是一种遗传性疾病,由亚铁螯合酶缺乏引起,该酶是血红素生物合成途径中的最后一种酶。大多数 EPP 患者表现为疼痛性光毒性的临床症状。肝损伤是 EPP 最严重的并发症,<5%的患者发生。本报告描述了一例 EPP 患者,其抱怨皮肤症状恶化、紧张和失眠。实验室检查显示红细胞中原卟啉浓度高度升高,血清转氨酶升高,提示与 EPP 相关的肝损伤。随后发现血清促甲状腺激素(TSH)降低,游离三碘甲状腺原氨酸(FT3)和游离甲状腺素(FT4)浓度升高,以及甲状腺过氧化物酶(TPO)和 TSH 受体抗体增加,导致 Graves 病的诊断。患者接受了 500MBq 的放射性碘(I(131))。放射性碘治疗 3 个月后,甲状腺体积减少至治疗前的 30%。尽管患者有轻微的甲状腺功能减退,但他的肝酶恢复正常,红细胞原卟啉浓度降低了五倍,皮肤症状显著改善。Graves 病和 EPP 同时存在是一种统计学上罕见的事件,除了我们的患者,文献中还报告了一例。虽然 Graves 病与 EPP 相互作用的确切机制尚待探索,但我们建议对有肝并发症的 EPP 患者进行甲状腺功能检查,以排除甲亢作为潜在病因。