Cryo-Depleted Plasma Infusions for Ligneous Conjunctivitis

This case report presents the clinical, histological, and genetic characteristics of a patient with ligneous conjunctivitis and to provide a long-term treatment schedule, using cryo-depleted plasma infusions in combination with topical cyclosporine as a successful therapeutic option.

A 12-year-old Turkish girl experienced years of ocular itching, irritation, and increased light sensitivity. Initial clinical examination revealed the presence of pseudomembranes and giant papillae on the tarsal conjunctivae of both eyes. The biopsy of a membrane from the left eye was taken for histological examination. Manifestations of severe comorbidity since infancy (congenital hydrocephalus, asthma, recurrent respiratory infections, and impaired wound healing) and parental consanguinity prompted a genetic evaluation. The pseudomembranes were composed of fibrin, granulation tissue, and inflammatory cells consistent with the diagnosis of ligneous conjunctivitis. The genetic analysis revealed homozygosity for a pathogenic missense variant in plasminogen (PLG), causing PLG deficiency. Due to severe systemic complications, the patient was successfully treated with cryo-depleted plasma infusions and cyclosporine eye drops, resulting in the complete remission of the ligneous conjunctivitis.

This case report highlights the importance of considering PLG deficiency in patients with recurring chronic conjunctivitis. Long-term treatment with cryo-depleted plasma infusions in combination with short-term use of topical cyclosporine may provide an efficient therapeutic option for ligneous conjunctivitis in patients with PLG deficiency.