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?
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