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

Abstract

Purpose: This review and case report address ligneous conjunctivitis (LC), a rare ocular condition caused by plasminogen deficiency type 1 (PLGD-1), which manifests as wood-like fibrin-rich membranes on the palpebral conjunctiva. The goal is to provide ophthalmologists-often the first physicians to encounter the condition-with a robust understanding of its systemic manifestations and to highlight current therapeutic strategies, with particular emphasis on the administration of intravenous plasminogen concentrate.

Methods: We present a clinical LC case alongside a narrative review of published cases, etiology, and treatment approaches.

Results: LC initially manifests as erythematous lesions on the palpebral conjunctiva, eventually developing into fibrinous, wood-like membranes. It is typically associated with PLGD-1, a genetic disorder of the plasminogen gene ( PLG ), which results in impaired fibrinolysis and the formation of fibrin deposits. LC often appears within the first year of life, but patients may experience a prolonged period before diagnosis. PLGD-1 is also associated with the development of mucosal lesions in many other organ systems. Contributing factors include chronic inflammation, allergens, infections, and trauma. There have been many proposed treatment approaches for LC, but the recent approval of intravenous plasma-derived human plasminogen concentrate replacement has been an important advance.

Conclusions: LC is an ocular disorder with potential vision- and life-threatening complications. The treatment of LC has historically been challenging; however, recognition of its association with PLGD-1 has led to more effective therapies, particularly intravenous plasminogen concentrate. Early diagnosis and coordinated care between ophthalmologists and other specialists, such as hematologists, are crucial to prevent complications, including vision loss.

Keywords: fibrin-rich membranes; ligneous conjunctivitis; ligneous membranes; plasminogen deficiency; plasminogen replacement therapy.