Ligneous conjunctivitis in a girl with severe type I plasminogen deficiency

Background: Ligneous conjunctivitis is a rare form of chronic recurrent pse udomembranous disease and may be associated with systemic membranous pathol ogical changes. Recently ligneous conjunctivitis has been linked to severe type I plasminogen deficiency. We report on a patient with plasminogen defi ciency and severe bilateral ligneous conjunctivitis. A new treatment approa ch and its outcome in this patient are described. Case report: We present the case of a 9-month-old Turkish girl with massive swelling of the eyelids and hard white pseudomembranes on both lids. The c onjunctival smear was positive for Streptococcus pneumoniae. The clinical d iagnosis was: ligneous conjunctivitis with superinfection. Histological inv estigation showed fibrin as major component of the pseudomembranes. The coa gulation analyses revealed decreased plasminogen activity (<5%; normal 80-1 20%) and decreased plasminogen antigen (<0.4 mg/dl; normal 6-25 mg/dl). The failure of surgical therapy led to the attempt at treatment with intraveno us lys-plasminogen. A significant improvement of the ocular symptoms occurr ed; stabilization with no recurrent pseudomembranes could be achieved for 6 months after treatment. Discussion: The initial amelioration of symptoms in our patient after syste mic replacement therapy confirms the etiological importance of plasminogen deficiency in the development of ligneous conjunctivitis. Curative treatmen t of ligneous conjunctivitis is still not available. However, intravenous a pplication of plasminogen offers new possibilities in therapy, although lon g-term treatment seems necessary.