loading...| Preferred Name |
迟发性皮肤卟啉症 / Porphyria cutanea tarda |
| Definitions |
Porphyria cutanea tarda (PCT) is due to an accumulation of uroporphyrins in plasma from blockage of the normal haem synthetic pathway in the liver at the level of uroporphyrinogen decarboxylase (URO-D). The majority of cases are sporadic and frequently associated with iron overload. PCT manifests as skin fragility and blistering in light-exposed skin, particularly on the dorsa of the hands, together with hypertrichosis. |
| ID |
http://purl.bmicc.cn/ontology/ICD11CN/5C58.10 |
| definition |
Porphyria cutanea tarda (PCT) is due to an accumulation of uroporphyrins in plasma from blockage of the normal haem synthetic pathway in the liver at the level of uroporphyrinogen decarboxylase (URO-D). The majority of cases are sporadic and frequently associated with iron overload. PCT manifests as skin fragility and blistering in light-exposed skin, particularly on the dorsa of the hands, together with hypertrichosis. |
| hasDbXref | |
| label |
迟发性皮肤卟啉症 / Porphyria cutanea tarda |
| mappingRelation | |
| notation |
5C58.10 |
| prefixIRI |
ICD11CN:C58.10 |
| prefLabel |
迟发性皮肤卟啉症 / Porphyria cutanea tarda |
| subClassOf |