Macdonald D M, Nicholson D C
Br J Dermatol. 1976 Aug;95(2):157-62. doi: 10.1111/j.1365-2133.1976.tb00819.x.
The terminal stages of erythropoietic protoporphyria are recorded. The observations are related to the site of the fundamental lesion and the nature of the biochemical defect. The possibly ominous prognosis in this usually mild condition is emphasized. Apart from congenital porphyria, the porphyrias do not usually confer severe cutaneous lesions. These diseases present to dermatologists because of moderate photosensitivity and are not usually regarded as a risk to life. Dangerous central nervous system involvement may occur, however, in acute intermittent, variegate and hereditary coproporphyrias, while in acquired symptomatic porphyria severe underlying liver dysfunction may occur. Probably the most common familial photosensitizing porphyria is erythropoietic protoporphyria. Recently some deaths from severe liver involvement have been reported in this disease.
记录了红细胞生成性原卟啉症的终末期。这些观察结果与根本病变的部位及生化缺陷的性质有关。强调了在这种通常为轻症的疾病中可能出现的不良预后。除先天性卟啉症外，卟啉症通常不会导致严重的皮肤病变。这些疾病因中度光敏感性而被皮肤科医生关注，通常不被视为对生命的威胁。然而，在急性间歇性、混合型及遗传性粪卟啉症中可能会出现危险的中枢神经系统受累，而在获得性症状性卟啉症中可能会出现严重的潜在肝功能障碍。红细胞生成性原卟啉症可能是最常见的家族性光致敏性卟啉症。最近有报道称该疾病出现了一些因严重肝脏受累导致的死亡病例。
