Hönigsmann H, Gschnait F, Konrad K, Stingi G, Wolff K
J Invest Dermatol. 1976 Mar;66(3):188-95. doi: 10.1111/1523-1747.ep12481938.
Albino mice were made protoporphyric with griseofulvin according to an established procedure. Photosensitivity flares were elicited once a week throughout a 10-month period, using black light as a source for 410 nm radiation and the flares were monitored by the intravenous injection of vascular tracers and by light and electron microscopy. Each irradiation led to a selective destruction of the endothelial cells of superficial capillaries which was followed by massive vascular leakage. The basal lamina remained largely intact, providing the scaffold for regenerating endothelial cells which deposited new basal lamina material at their periphery. Subsequent exposures to 410 nm radiation reproduced the endothelial damage and subsequent basal lamina formation; multiple irradiations thus resulted in excessive, concentric, tubelike basal lamina deposits around dermal vessels which light microscopically appeared as PAS-positive hyaline material and clinically gave the skin a thickened, waxy appearance. This model thus reproduced the skin of erythropoietic protoporphyria clinically, microscopically, and at the ultrastructural level.
根据既定程序，用灰黄霉素使白化小鼠产生原卟啉。在10个月的时间里，每周一次引发光敏性皮疹，使用黑光作为410nm辐射源，并通过静脉注射血管示踪剂以及光镜和电镜观察来监测皮疹。每次照射都会导致浅表毛细血管内皮细胞的选择性破坏，随后出现大量血管渗漏。基膜基本保持完整，为再生的内皮细胞提供支架，这些内皮细胞在其周边沉积新的基膜物质。随后暴露于410nm辐射会再次造成内皮损伤和随后的基膜形成；多次照射会导致真皮血管周围出现过多、同心的管状基膜沉积物，在光镜下表现为PAS阳性透明物质，临床上使皮肤呈现增厚、蜡样外观。因此，该模型在临床、显微镜和超微结构水平上再现了红细胞生成性原卟啉症的皮肤表现。
