Congenital plasminogen deficiency Type 1 may first be recognized by a bilateral conjunctivitis associated with abnormal fibrinolysis amd accumulation of subepithelial fibrin..


Chromosome location: 6q26

Gene: PLG


Inheritance: autosomal recessive


Clinical findings:

(1) ligneous conjunctivitis, with bilateral pseudomembranes on the tarsal conjunctiva with a wood-like consistency that may be white, yellowish or reddish in color

(2) similar lesions develop on the mucous membranes such as the mouth, nasopharynx, trachea and genital tract

(3) impaired wound healing of mucuous membranes

(4) variable congenital occlusive hydrocephalus

(5) variable juvenile colloid milium


Laboratory findings:

(1) decreased serum plasminogen activity with decreased fibrinolysis

(2) decreased serum concentration of plasminogen antigen


The condition is not associated with increased thormboembolic events.


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