" /> Porphyria cutanea tarda - CISMeF





ICD-11 code : 5C58.10;

Preferred Label : Porphyria cutanea tarda;

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.;

ICD-11 synonym : UROD deficiency; UROD - [uroporphyrinogen decarboxylase] deficiency; Porphyria, hepatocutaneous type; Heterozygous uroporphyrinogen decarboxylase deficiency; PCT - [porphyria cutanea tarda];

ICD-11 acronym : PCT;

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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.

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05/05/2025


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