Catalog

Porphyria cutanea tarda

5C58.10

Link to WHO Definition

Porphyria cutanea tarda – http://id.who.int/icd/entity/370983230

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.

Parents

Children