5C58.13

ICD-11 MMS 2026-01

Variegate porphyria

Description

Variegate porphyria is a form of acute hepatic porphyria characterised by the occurrence of neuro-visceral attacks with or without the presence of cutaneous lesions (bullous photodermatitis).

Index terms

  • Variegate porphyria
  • Porphyria variegata
  • Protocoproporphyria
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  • Protoporphyrinogen oxidase deficiency
  • VP - [variegate porphyria]

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

  • 5C58.10 – Porphyria cutanea tarda
  • 5C58.12 – Erythropoietic porphyrias
  • 5C58.1Y – Other specified porphyrias
  • 5C58.1Z – Porphyrias, unspecified

Foundation entity

Variegate porphyria – http://id.who.int/icd/entity/1227474618

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