title: Adult-Onset Ligneous Conjunctivitis with Detection of a Novel Plasminogen Gene Mutation and Anti-Plasminogen IgA Antibody: A Clinicopathologic Study and Review of Literature.
abstract: OBJECTIVE
  To report a novel plasminogen gene mutation and detection of anti-plasminogen antibodies in a patient with ligneous conjunctivitis successfully treated with 60% fresh frozen plasma (FFP).
  
  
  METHODS
  Retrospective data collected on a 45-year-old Caucasian female presenting with unilateral chronic membranous lesions.
  
  
  RESULTS
  Laboratory investigation demonstrated decreased plasminogen antigen level, plasminogen activity, and rate of plasminogen activation by u-PA or t-PA, and elevated plasminogen activator inhibitor-1. Anti-plasminogen IgG and IgA antibodies were detected. DNA analysis revealed a novel Asp432Asn heterozygous missense mutation in the plasminogen gene (exon 11). The patient was treated with topical 60% FFP, achieved complete remission after four months, and remained membrane-free for over five years of follow-up.
  
  
  CONCLUSIONS
  A novel plasminogen gene mutation, deficiency of plasminogen antigen and activity, and anti-plasminogen IgG and IgA antibodies were identified in a patient with adult-onset ligneous conjunctivitis. Sixty percent FFP maintained this patient disease-free for over five years.
authors: a The Institute of Ophthalmology and Visual Science, New Jersey Medical School, Rutgers University , Newark , NJ , USA .
authors: Yufei
authors: Y
authors: Tu
authors: b Department of Pathology , Duke University , Durham , NC , USA .
authors: Mario
authors: M
authors: Gonzalez-Gronow
authors: c The Department of Ophthalmology , University of Pittsburgh Medical Center , Pittsburgh , PA , USA .
authors: Anton M
authors: AM
authors: Kolomeyer
authors: d The Frederick B. Cohen Comprehensive Cancer and Blood Disorders Center, Newark Beth Israel Medical Center , Newark , NJ , USA .
authors: Alice
authors: A
authors: Cohen
authors: a The Institute of Ophthalmology and Visual Science, New Jersey Medical School, Rutgers University , Newark , NJ , USA .
authors: Joanna
authors: J
authors: Pruzon
authors: e The New York Eye and Ear Infirmary , New York , NY , USA , and.
authors: Tatyana
authors: T
authors: Milman
authors: a The Institute of Ophthalmology and Visual Science, New Jersey Medical School, Rutgers University , Newark , NJ , USA .|f Metropolitan Eye Research and Surgery Institute , Palisades Park , NJ , USA.
authors: David S
authors: DS
authors: Chu
mesh_terms: D003231:Conjunctivitis / Q000235:genetics* / Q000276:immunology* / Q000628:therapy; D004252:DNA Mutational Analysis; D004797:Enzyme-Linked Immunosorbent Assay; D005091:Exons / Q000235:genetics; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood*; D007074:Immunoglobulin G / Q000097:blood; D008875:Middle Aged; D020125:Mutation, Missense; D010949:Plasma; D010958:Plasminogen / Q000172:deficiency* / Q000235:genetics / Q000276:immunology*; D016133:Polymerase Chain Reaction; D012189:Retrospective Studies; D012873:Skin Diseases, Genetic / Q000235:genetics* / Q000276:immunology*
chemical_list: D007070:Immunoglobulin A; D007074:Immunoglobulin G; D010958:Plasminogen
keywords: Anti-plasminogen antibody; fresh frozen plasma; ligneous conjunctivitis; plasminogen