5C58.10

ICD-11 MMS 2026-01

Porphyria cutanea tarda

Description

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.

Index terms

  • Porphyria cutanea tarda
  • Heterozygous uroporphyrinogen decarboxylase deficiency
  • PCT - [porphyria cutanea tarda]
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  • Porphyria, hepatocutaneous type
  • UROD - [uroporphyrinogen decarboxylase] deficiency
  • Sporadic porphyria cutanea tarda
  • Sporadic PCT - [porphyria cutanea tarda]
  • Familial porphyria cutanea tarda
  • Familial PCT - [porphyria cutanea tarda]
  • Toxic porphyria cutanea tarda
  • Porphyria turcica

Exclusions from above levels

  • http://id.who.int/icd/entity/1191191920 – Disorders of lipoprotein metabolism or certain specified lipidaemias
  • 3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
  • 5A71.01 – Congenital adrenal hyperplasia
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  • 5A81.1 – 5-alpha-reductase deficiency
  • LD28.01 – Marfan syndrome
  • LD28.1 – Ehlers-Danlos syndrome
  • LD2A.4 – androgen resistance syndrome
  • 18 – Pregnancy, childbirth or the puerperium
  • http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

  • 5C58.12 – Erythropoietic porphyrias
  • 5C58.13 – Variegate porphyria
  • 5C58.1Y – Other specified porphyrias
  • 5C58.1Z – Porphyrias, unspecified

Foundation entity

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

Part of: 5C58.1: Porphyrias › 5C58: Inborn errors of porphyrin or heme metabolism › 05: Endocrine, nutritional or metabolic diseases