[红细胞生成性原卟啉病]
[Erythropoietic protoporphyria].
作者信息
de Sélys R, Decroix J, Frankart M, Hassoun A, Willocx D, Pirard C, Bourlond A
机构信息
Service de Dermatologie, Faculté de Médecine, Cliniques universitaires Saint-Luc, Bruxelles.
出版信息
Ann Dermatol Venereol. 1988;115(5):555-60.
The investigation and follow-up of three cases of EPP led us to reevaluate the signs and symptoms of the disease, the usefulness of early skin biopsies and protoporphyrin and ferrochelatase determinations. A review of the literature brought forth the salient features of this inborn error of porphyrin metabolism and stressed the value of treatment with beta-carotene and canthaxantine but also the risk of the so-called "gold dust" retinopathy.
对三例红细胞生成性原卟啉病（EPP）病例的调查及随访，促使我们重新评估该病的体征和症状、早期皮肤活检以及原卟啉和亚铁螯合酶测定的作用。文献回顾揭示了这种卟啉代谢先天性异常的显著特征，并强调了β-胡萝卜素和角黄素治疗的价值，但也指出了所谓“金粉”视网膜病变的风险。
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