Ligneous conjunctivitis in a patient with plasminogen type I deficiency - case report with review of literature

Background: Ligneous conjunctivitis is a rare chronic pseudomembranous conjunctivitis. It forms nodular masses on the palpebral conjunctiva. Beside the conjunctival affection pseudomembranes can also be found on other mucosal tissues. Patient: We report on a male baby who had a ventriculoperitoneal shunt due to hydrocephalus internus on his fourth day after birth. Recurrent pseudomebranous conjunctivitis started in the First week of life. This was refractory to drug therapy and reoccurred soon after surgical procedure. The analysis of clotting parameters revealed homozygous plasminogen deficiency. Conclusion: Recently type 1 plasminogen deficiency seems to be a major reason for developing conjunctivitis lignosa. Homozygous and heterozygous mutations in the plasminogen gene are found. At the moment no satisfactory therapy is available. Cases with mild ophthalmological symptoms seem to be positively influenced by a therapy of topical steroids combined with heparin, In severe cases with generalised symptoms systemic therapy with lys-plasminogen is necessary. High costs and poor bioavailability complicate systemic therapy. Improved plasminogen variants for treatment of severe type 1 plasminogen deficiency would be of great clinical importance.