Porphyria cutanea tarda (Q40111): Difference between revisions
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A porfiria cutânea tardia é devido a um acúmulo de uroporfirinas no plasma devido ao bloqueio da via sintética normal do heme no fígado ao nível da uroporfirinogênio descarboxilase (URO-D). A maioria dos casos é esporádica e freqüentemente associada à sobrecarga de ferro. Ela se manifesta como fragilidade da pele e bolhas na pele exposta à luz, principalmente no dorso das mãos, juntamente com hipertricose. | |||
| description / en | description / en | ||
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. | |||
Revision as of 05:47, 13 August 2026
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.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | 5C58.10 |
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| English | Porphyria cutanea tarda |
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. |
