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Porphyria variegata

Disease definition

Variegate porphyria is a form of acute hepatic porphyria (see this term) characterized by the occurrence of neuro-visceral attacks with or without the presence of cutaneous lesions.


Classification level: Disorder
  • Synonym(s):
    • Protoporphyrinogen oxidase deficiency
    • Variegate porphyria
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Adolescent, Adult
  • ICD-10: E80.2
  • OMIM: 176200
  • UMLS: C0162532
  • MeSH: -
  • GARD: 7848
  • MedDRA: -

Detailed information


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