Figure 1.

Patients with congenital plasminogen deficiency have immunopathologies at many mucosal barrier sites. (A) Congenital plasminogen deficiency (CPD) is associated with abnormal accumulation of an amorphous, fibrin- and leukocyte-rich, amyloid-like substance in the lamina propria of mucous membranes, including the oral mucosa, the eyes, respiratory system, urinary and genital systems, and the gastrointestinal system. (B) Plasminogen (PLG)–deficient mice display an array of mucosal immunopathologies phenocopying the human disease, including ligneous conjunctivitis; periodontitis; osteoporosis; systemic inflammation, as denoted by enlarged submandibular lymph nodes (SMLN) and splenomegaly; and inflammation of the gastrointestinal tract—stomach ulcers, colitis-like phenotype, and rectal prolapse.