Ligneous Conjunctivitis With Ear Involvement

Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency

“…An inflammatory reaction combined with activation of inflammatory cells and fibroblasts, with drying out of the fibrin, results in the wood-like appearance of the conjunctival lesions [22]. In many patients with ligneous conjunctivitis, additional similar pseudomembranous lesions were observed in the mucosa of the mouth, tongue, nasopharynx, tracheobronchial tree, and female genital tract [13,16,20,27]. Some children with ligneous conjunctivitis had also congenital occlusive hydrocephalus [19,22] which could be also observed in plasminogen-deficient mice [8].…”

“…The severe forms of type I plasminogen deficiency may cause ligneous conjunctivitis, an unusual and rare form of chronic conjunctivitis characterised by pseudomembranes on the palpebral surfaces [18]. Furthermore, pseudomembranous lesions of other mucous membranes in the mouth, nasopharynx, tracheobronchoalveolar tree, and female genital tract as well as congenital occlusive hydrocephalus have been reported in patients with type I plasminogen deficiency [13,16,20,27].…”

Spontaneous development of otitis media in plasminogen-deficient mice

“…Ligneous conjunctivitis is a rare condition characterized by the formation of fibrin-rich viscous material on the conjunctiva and the mucous membranes of the upper and lower airways (Schuster and Seregard, 2003). Involvement of the ear in patients with ligneous conjunctivitis, called ligneous otitis, is associated with fibrin deposition in the middle ear (Chai and Coates, 2003;Hyden et al, 2002;Marcus et al, 1990). The ears from these patients showed an accumulation of an amorphous, wood-like material in the MEC.…”

Therapy with a Purified Plasminogen Concentrate in an Infant with Ligneous Conjunctivitis and Homozygous Plasminogen Deficiency

“…The disease is frequently associated with nasopharyngitis, tracheobronchial obstruction, otitis media, vulvovaginitis, and defective wound healing. [2][3][4][5][6][7][8][9] Pseudomembranous conjunctivitis was first described in 1847 by Bouisson,10 and the term "conjunctivitis lignosa" was introduced by Borel in 1933. 11 More than 100 cases have been reported in the literature, but no satisfactory treatment has yet been found.…”