Nonaka S, Takamiyagi A, Maruno M
Department of Dermatology, Faculty of Medicine, University of the Ryukyus.
Nihon Rinsho. 1995 Jun;53(6):1449-55.
Porphyrins are fluorescent chemicals excited by visual light. Skin changes in patients with porphyrias develop when the skin is exposed to sunlight and porphyrins have precipitated. This reaction is phototoxic. Since the phototoxic reaction varies with each porphyria, each porphyria demonstrates different skin changes. Conversely, skin changes do not develop if skins are completely protected from exposure to the sun in patients with porphyrias with show severe abnormalities in the metabolism of porphyrin pathways. In Japan, relatively popular porphyrias in dermatology clinic are porphyria cutanea tarda (PCT), erythropoietic protoporphyria (EPP) and congenital erythropoietic porphyria (CEP). Histopathologically, one of the most important findings in porphyria is a deposition of PAS positive materials at the dermo-epidermal junction and around the small blood vessels in the dermis.
卟啉是由可见光激发的荧光化学物质。卟啉症患者的皮肤变化是在皮肤暴露于阳光且卟啉沉淀时发生的。这种反应具有光毒性。由于光毒性反应因每种卟啉症而异，每种卟啉症都表现出不同的皮肤变化。相反，在卟啉代谢途径存在严重异常的卟啉症患者中，如果皮肤得到完全保护不暴露于阳光，则不会出现皮肤变化。在日本，皮肤科诊所中相对常见的卟啉症有迟发性皮肤卟啉症（PCT）、红细胞生成性原卟啉症（EPP）和先天性红细胞生成性卟啉症（CEP）。组织病理学上，卟啉症最重要的发现之一是在真皮表皮交界处和真皮小血管周围有PAS阳性物质沉积。
