%0 Case Reports
%T Adult-Onset Ligneous Conjunctivitis with Detection of a Novel Plasminogen Gene Mutation and Anti-Plasminogen IgA Antibody: A Clinicopathologic Study and Review of Literature.
%A Tu Y
%A Gonzalez-Gronow M
%A Kolomeyer AM
%A Cohen A
%A Pruzon J
%A Milman T
%A Chu DS
%J Semin Ophthalmol
%V 31
%N 6
%D 2016
%M 25674820
%F 2.246
%R 10.3109/08820538.2015.1005319
%X 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.