Conjunctivitis, Ligneous

Clinical Characteristics

Ocular Features:

The most common clinical feature of this disorder is conjunctivitis often precipitated by an injury or infection of the conjunctiva with typical inflammatory features. A thick, ligneous (wood-like) pseudomembrane containing clotted fibrin subsequently forms that appears white, yellow-white, or finally red. These can be surgically removed but tend to recur, often on an accelerated timescale. This process is most pronounced on the upper tarsal conjunctiva but often involves the bulbar conjunctiva and the cornea as well. Corneal involvement (in nearly a third of cases) can lead to vascularization, scarring, keratomalacia, and perforation. The disease may be intermittent.

Systemic Features:

While the conjunctiva is most commonly involved, other mucosal surfaces such as oral, laryngeal, tracheal, and vaginal mucosas may have similar disease often concomitantly with the ocular lesions. Ligneous gingivitis consists of nodular lesions with ulceration and peridodontal tissue destruction. The ear may also be involved in the disease. Life-threatening airway obstruction is a threat, especially in children during acute recurrences. Occlusive hydrocephalus requiring shunting occurs in a significant number of affected children.

Genetics

This autosomal recessive condition seems to result from homozygous mutations in the PLG [1] gene (6q26) that codes for plasminogen. Tears contain plasminogen activators that convert plasminogen into the fibrinolytic enzyme plasmin which normally clears conjunctival and corneal fibrin deposits. The lack of normal plasminogen allows fibrin to accumulate on all mucosal surfaces.

Treatment

Treatment Options:

Four weeks of treatment with IV human Glu-plasminogen in a small group of children and adults with congenital plasminogen deficiency produced marked clinical improvement in ocular as well as disseminated symptoms according to a recent report.

Surgical excision of the pseudomembrane is possible but multiple recurrences are common. Spontaneous resolution has been reported.

References

Article Title:

Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency [2]

Shapiro AD, Nakar C, Parker JM, Albert GR, Moran JE, Thibaudeau K, Thukral N, Hardesty BM, Laurin P, Sandset PM. Plasminogen replacement therapy for the treatment of children and adults with congenital plasminogen deficiency. Blood. 2018 Mar 22;131(12):1301-1310.

PubMed ID:

29321155

Treatment of plasminogen deficiency patients with fresh frozen plasma [3]

Kizilocak H, Ozdemir N, Dikme G, Koc B, Atabek AA, Cokugras H, Iskeleli G, Donmez-Demir B, Christiansen NM, Ziegler M, Ozdag H, Schuster V, Celkan T. Treatment of plasminogen deficiency patients with fresh frozen plasma. Pediatr Blood Cancer. 2017 Sep 6. doi: 10.1002/pbc.26779. [Epub ahead of print].

PubMed ID:

28876531

Destructive Membranous Periodontal Disease (Ligneous Gingivitis) [4]

Sivolella S, De Biagi M, Sartori MT, Berengo M, Bressan E. Destructive Membranous Periodontal Disease (Ligneous Gingivitis): A Literature Review. J Periodontol. 2011 Aug 22. [Epub ahead of print)

PubMed ID:

21859319

Ligneous conjunctivitis [5]

Schuster V, Seregard S. Ligneous conjunctivitis. Surv Ophthalmol. 2003 Jul-Aug;48(4):369-88. Review.

PubMed ID:

12850227

Therapy with a purified plasminogen concentrate in an infant with ligneous conjunctivitis and homozygous plasminogen deficiency [6]

Schott D, Dempfle CE, Beck P, Liermann A, Mohr-Pennert A, Goldner M, Mehlem P, Azuma H, Schuster V, Mingers AM, Schwarz HP, Kramer MD. Therapy with a purified plasminogen concentrate in an infant with ligneous conjunctivitis and homozygous plasminogen deficiency. N Engl J Med. 1998 Dec 3;339(23):1679-86.

PubMed ID:

9834305