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Ligneous Conjunctivitis
Key Concepts
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Ligneous conjunctivitis is a rare disorder associated with systemic plasminogen deficiency.
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Only type I hypoplasminogenemia (reduction in plasminogen level and activity) has been shown to cause any form of pseudomembranous disease.
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The most common manifestation of systemic plasminogen deficiency is chronic conjunctivitis, which can produce true membranous lesions that are highly vascularized, raised, and friable.
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Plasminogen-deficient patients can have systemic symptoms involving the respiratory tract, gingiva, ears, urogenital tract, gastrointestinal tract, central nervous system, and skin.
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Treatment of ligneous conjunctivitis is based on plasminogen substitution; at present, however, there is no standard treatment regimen.
Ligneous conjunctivitis (LC) is a rare disorder associated with systemic plasminogen deficiency and characterized by a protracted course of recurrent membranous conjunctival lesions. This ocular manifestation of a systemic disorder typically presents in childhood, although it can occur at any age. It may be associated with lesions of other mucous membranes including those of the mouth, ear, nasopharynx, trachea and respiratory tract, gastrointestinal tract, and urogenital tract. Rarely, it is seen in conjunction with congenital occlusive hydrocephalus or juvenile colloid milium.
In 1847, Bouisson described a 46-year-old man with bilateral pseudomembranous conjunctivitis in what is thought to be the first published case of LC. In 1933, Borel assigned the name ligneous , meaning “woody,” to this disorder because of the characteristic wood-like consistency of the membranes in severe cases. Most patients under active treatment have thinner, softer membranous lesions ( Fig. 49.1 ).
Ligneous conjunctivitis of the superior palpebral conjunctiva. Note the white, avascular lesion at the perimeter and the vascularized base.
Epidemiology
LC is rare. Since the introduction of the term, more than 209 cases have been described in the literature. In a series of 23 patients with severe type I plasminogen deficiency, data were collated to report on these newly described patients and all previously published cases. The median age of first clinical manifestation was 1 year (the mean age at onset was 5 years and 5 months), with a female-to-male ratio of 1.55:1. For unknown reasons, LC has been reported in the literature to be more common in females than in males. , The most common manifestation in this group was LC (87%), followed by respiratory tract involvement (33%) and ligneous gingivitis/periodontitis (32%). More rare extraocular manifestations included involvement of the ears, the female genital tract (19%), the male urogenital tract (2%), the gastrointestinal tract (3%), hydrocephalus (14%), Dandy-Walker malformation (6%), and juvenile colloid milium of the skin. One recent case report found LC associated with systemic IgG4-related disease and another with Lyell syndrome, which is of immune-allergic origin. , Several studies have shown extraocular manifestations of severe plasminogen deficiency without ocular involvement. , ,
Numerous case reports have described this condition after infection or trauma. An abnormal inflammatory response to conjunctival injury is likely a factor in the development of this disease. Of interest are cases that occurred after surgery to the conjunctiva, including surgery for pterygium, , pingueculum, strabismus, , cataract, keratoplasty, ptosis, and conjunctival autograft from a healthy donor eye for the fellow eye, which had LC. The induction of bilateral LC has even been seen with use of an ocular prosthesis in an anophthalmic socket.
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