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迟发性皮肤卟啉病。诊断、管理及与其他肝性卟啉病的鉴别。

Porphyria cutanea tarda. Diagnosis, management, and differentiation from other hepatic porphyrias.

作者信息

Grossman M E, Poh-Fitzpatrick M B

出版信息

Dermatol Clin. 1986 Apr;4(2):297-309.

PMID:3955900
Abstract

Porphyria cutanea tarda is a photocutaneous syndrome characterized clinically by cutaneous fragility, bullae, hypertrichosis, pigmentary changes, and sclerodermoid plaques and characterized biochemically by hepatic overproduction and storage of excessive amounts of porphyrins. Porphyria cutanea tarda, the most common disorder of porphyrin metabolism, must be differentiated from variegate porphyria, hereditary coproporphyria, bullous dermatosis of hemodialysis, and drug-related pseudoporphyria.

摘要

迟发性皮肤卟啉病是一种光皮肤综合征,临床特征为皮肤脆弱、水疱、多毛症、色素沉着改变和硬皮病样斑块,生化特征为肝脏卟啉过度生成和过量储存。迟发性皮肤卟啉病是卟啉代谢最常见的疾病,必须与杂合性卟啉病、遗传性粪卟啉病、血液透析大疱性皮肤病和药物相关假性卟啉病相鉴别。

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