ICD-11 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.;
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.