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Hypoplasminogenemia

ORPHA:722· ICD-10 L90.5

Definition

A rare multi-system disease characterized by markedly impaired extracellular fibrinolysis leading to the formation of ligneous (fibrin-rich) pseudomembranes on mucosae.

Prevalence
1-9 / 1 000 000
Inheritance
Autosomal recessive
Age of onset
All ages