Advancing Understanding of Ligneous Conjunctivitis: Bridging Pathogenesis, Diagnosis, and Therapy

Area of Science:

• Ophthalmology
• Genetics
• Hematology

Background:

• Ligneous conjunctivitis (LC) is a rare condition characterized by the formation of thick, wood-like, fibrin-rich membranes on the palpebral conjunctiva.
• It is primarily caused by plasminogen deficiency type 1 (PLGD-1), a genetic disorder affecting the plasminogen gene (PLG).
• PLGD-1 impairs fibrinolysis, leading to fibrin deposition and the characteristic conjunctival membranes, often presenting in infancy.

Purpose of the Study:

• To provide ophthalmologists with a comprehensive understanding of ligneous conjunctivitis (LC).
• To review the systemic manifestations of LC associated with plasminogen deficiency type 1 (PLGD-1).
• To highlight current and emerging therapeutic strategies for LC, emphasizing plasminogen concentrate replacement therapy.

Main Methods:

• A narrative review of published literature on ligneous conjunctivitis (LC).
• Inclusion of a clinical case report detailing the presentation, diagnosis, and management of LC.
• Analysis of etiology, clinical features, and treatment approaches for LC.

Main Results:

• LC presents as erythematous lesions progressing to fibrinous, wood-like membranes on the conjunctiva.
• Plasminogen deficiency type 1 (PLGD-1) is the underlying genetic cause, leading to impaired fibrinolysis and fibrin deposits.
• While LC can occur in infancy, diagnosis may be delayed; systemic mucosal lesions are also associated with PLGD-1.

Conclusions:

• Ligneous conjunctivitis (LC) poses significant risks to vision and potentially life, necessitating prompt recognition and management.
• The association with plasminogen deficiency type 1 (PLGD-1) has paved the way for more effective treatments, notably intravenous plasminogen concentrate.
• Early diagnosis and multidisciplinary care involving ophthalmologists and hematologists are essential to prevent severe complications, including vision loss.