Orphanet: Ipoplasminogenemia

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Disease definition

Severe hypoplasminogenemia (HPG) or type 1 plasminogen (plg) deficiency is a systemic disease characterised by markedly impaired extracellular fibrinolysis leading to the formation of ligneous (fibrin-rich) pseudomembranes on mucosae during wound healing.


Classification level: Disorder
  • Synonym(s):
    • Plasminogen deficiency type 1
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive or Not applicable 
  • Age of onset: All ages
  • ICD-10: L90.5
  • OMIM: 217090
  • UMLS: C0398621  C1968804
  • MeSH: -
  • GARD: 4380
  • MedDRA: -
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