Nonaka S, Takamiyagi A
Department of Dermatology, Faculty of Medicine, University of the Ryukyus.
Nihon Rinsho. 1995 Jun;53(6):1427-32.
Porphyria cutanea tarda (PCT) is induced by an enzyme deficiency of hepatic uroporphyrinogen decarboxylase activity. As the result of this enzyme deficiency, uroporphyrin and coproporphyrin accumulate to the skin and other organs. These porphyrins are excreted into urine because of their easy solubility in water. Exposure to the sunlight of the porphyrin-rich skin induces cutaneous changes. Porphyria cutanea tarda develops mainly in middle-aged males, sometimes in females. Ethylalcohol, estrogenic hormones, and hemodialysis are reported as provocative factors. Hyperpigmentation on exposed areas, skin fragility, vesicles and erosions are common in PCT. Histopathologically, subepidermal bulla is a characteristic finding in PCT. PAS positive materials are also prominent around the small blood vessels in the dermis and dermo-epidermal junctions. Hepatoerythropoietic porphyria (HEP) is usually manifest in early childhood with dark urine and reveals severe cutaneous photosensitivity. This disease is a homozygous form of type II PCT.
迟发性皮肤卟啉症（PCT）由肝脏尿卟啉原脱羧酶活性的酶缺乏引起。由于这种酶缺乏，尿卟啉和粪卟啉在皮肤和其他器官中积累。这些卟啉因其易溶于水而排泄到尿液中。富含卟啉的皮肤暴露于阳光下会引起皮肤变化。迟发性皮肤卟啉症主要发生在中年男性，有时也发生在女性。乙醇、雌激素和血液透析被报道为诱发因素。暴露部位色素沉着、皮肤脆弱、水疱和糜烂在PCT中很常见。组织病理学上，表皮下大疱是PCT的特征性表现。真皮和真皮-表皮交界处的小血管周围PAS阳性物质也很突出。肝红细胞生成性卟啉症（HEP）通常在幼儿期表现为尿液颜色深，并表现出严重的皮肤光敏感性。这种疾病是II型PCT的纯合形式。
