一种肝卟啉合成障碍的慢性疾病，好发于40-70岁，人群发病率<1%，占所有卟啉病1/3，男女比为2：1。
6.2.2 迟发性皮肤卟啉病
定义
病因和发病机理
分为遗传性(常染色体显性遗传) 或获得性： 尿卟啉原-III 脱羧酶缺陷。
¤ 诱因：酒精、药物（如抗真菌药、镇痛药、巴比托、雌激素和氯喹）、中毒（如铅、砷和六氯苯处理的小麦），以及肝脏感染性疾病，如HIV合并 HCV。
实验室检查
血液：血清铁水平增高，肝酶升高
尿液：尿卟啉水平增高 (I > III)
粪便：卟啉水平增高
组织病理
表皮下水疱，基底膜和血管壁增厚。
鉴别诊断
需和其他水疱性疾病相鉴别。
治疗
¤ 局部治疗：避光剂 (SPF/LSF>30)。
¤ 系统治疗：避免诱因 (如酒精和肝毒性药物)，放血疗法可降低体内铁储存，小剂量氯喹。
Tests
- This 60-year-old wine dealer complains of sores and bruises on the backs of his hands. What tests are indicated?
- This 30-year-old beautician with known hepatitis C infection complains of increased facial hair requiring epilation and an eczema on the backs of her hands. What is the most likely diagnosis?
- Statement 1 A positive Nikolski sign is typical for porphyria cutanea tarda
- Which additional studies are appropriate in porphyria cutanea tarda?
- Which statement about porphyria cutanea tarda is false?
- Statement 1 On Wood light examination, the urine in porphyria cutanea tarda fluoresces red-orange.
- Which of these treatments are appropriate for porphyria cutanea tarda?
Comments
Be the first one to leave a comment!