Plasminogen deficiency type 1

Plasminogen deficiency type 1, also known as hypoplasminogenemia, is an ultrarare autosomal recessive multisystem disorder caused by mutations in the *PLG* gene It is characterized pathophysiologically by markedly reduced levels of both immunoreactive plasminogen antigen and functional plasminogen activity, leading to severely impaired extracellular fibrinolysis and inadequate wound healing Clinically, this defect results in the spontaneous or trauma-induced accumulation of fibrin and chronic in...

Plasminogen deficiency type 1, also known as hypoplasminogenemia, is an ultrarare autosomal recessive multisystem disorder caused by mutations in the *PLG* gene It is characterized pathophysiologically by markedly reduced levels of both immunoreactive plasminogen antigen and functional plasminogen activity, leading to severely impaired extracellular fibrinolysis and inadequate wound healing

Plasminogen deficiency type 1, also known as hypoplasminogenemia, is an ultrarare autosomal recessive multisystem disorder caused by mutations in the *PLG* gene It is characterized pathophysiologically by markedly reduced levels of both immunoreactive plasminogen antigen and functional plasminogen activity, leading to severely impaired extracellular fibrinolysis and inadequate wound healing

Clinically, this defect results in the spontaneous or trauma-induced accumulation of fibrin and chronic inflammation across mucosal membranes, producing dense, "woody" pseudomembranous lesions While ligneous conjunctivitis is the most hallmark presentation and can lead to corneal scarring and vision loss, lesions frequently compromise the respiratory tract, oral cavity, gastrointestinal tract, and genitourinary system

Clinically, this defect results in the spontaneous or trauma-induced accumulation of fibrin and chronic inflammation across mucosal membranes, producing dense, "woody" pseudomembranous lesions