Therapeutic approaches in the treatment of ligneous conjunctivitis, the most common clinical manifestation of a systemic disease, caused by severe type I plasminogen deficiency

2004 ◽  
Vol 138 (4) ◽  
pp. 699 ◽  
Author(s):  
Katrin Tefs ◽  
Boris Hügle ◽  
Volker Schuster
2003 ◽  
Vol 90 (07) ◽  
pp. 86-91 ◽  
Author(s):  
Graziella Saggiorato ◽  
Donatella Pellati ◽  
Alessandra Casonato ◽  
Luca Spiezia ◽  
Elena Pontara ◽  
...  

SummarySevere type I plasminogen deficiency is the underlying cause of ligneous conjunctivitis, a rare disease characterized by wood-like pseudomembranes developing on the ocular and extraocular mucosa. Two unrelated female patients with ligneous conjunctivitis and moderate hypoplasminogenemia are described. Being of fertile age, they were treated with oral contraceptives, which determined a marked increase in plasminogen levels. Moreover, a palpebral pseudomembrane stopped growing in one patient and disappeared completely in the other while on the estroprogestinic treatment. In patient n.2,who also suffered from von Willebrand’s disease, prior Cushing’s disease induced an increase in both von Willebrand factor and plasminogen levels, which dropped after curative hypophysectomy. Genetic plasminogen study showed a 19Lys>Glu mutation in a heterozygous state in the first proposita and in a homozygous state in the second proband. In addition, both index patients were homozygous for a new intron F-14T>G mutation, which was found to reduce the acceptor splicing site prediction score.In conclusion, oral contraceptive therapy may improve plasminogen deficiency and deserves attention as an alternative therapeutic approach in selected cases of ligneous conjunctivitis with low, but not absent, plasminogen synthesis.


2021 ◽  
pp. 112067212110183
Author(s):  
Dimitris Dimopoulos ◽  
Andreas Zacharioudakis ◽  
Georgios Kazamias ◽  
Viasiliki Danilatou ◽  
Dimitra Katerini ◽  
...  

Ligneous conjunctivitis is a rare form of chronic, recurrent conjunctivitis characterized by wood-like, fibrinous pseudomembranes, which may be associated with systemic disease manifestations. It has been associated with congenital plasminogen (PLG) deficiency that is inherited with an autosomal recessive pattern due to mutations in the PLG gene and a variety of other genes, leading to disturbed wound healing. In this case report, we present the clinical, laboratory, and histopathological findings of a 36-year-old female patient who presented at the ophthalmology department with complaints of redness, irritation for the previous few weeks, and appearance of membranous lesions mainly on the tarsal conjunctivae. During biomicroscopic examination we found thick, yellowish-white pseudomembranes, and conjunctival proliferation with ligneous induration on the conjunctiva, located on the upper eyelids. Histopathological evaluations showed up ligneous conjunctivitis and laboratory evaluation confirmed a severe plasminogen deficiency (PLG < 2%). The patient was treated with topical fresh frozen plasma (FFP), topical steroids, heparin eye drops, and artificial tear drops daily, without systemic therapy.


2001 ◽  
Vol 85 (06) ◽  
pp. 1004-1010 ◽  
Author(s):  
Petra Zeitler ◽  
Stefan Seregard ◽  
Ugur Ozcelik ◽  
Deniz Anadol ◽  
Lori Luchtman-Jones ◽  
...  

SummarySevere type I plasminogen deficiency has been recently linked to ligneous conjunctivitis, a rare and uncommon form of chronic conjunctivitis. In this study, eight unrelated ligneous conjunctivitis patients living in different parts of the world were examined. All affected subjects from which plasma was available displayed absent or markedly reduced plasminogen antigen and plasminogen functional activity. Molecular genetic studies of seven patients identified a Lys19 → Glu mutation in two boys in a homozygous state, and in two girls in a compound-heterozygous state in which the second plasminogen gene carried a missense (Arg134 → Lys) and a nonsense mutation (Cys133 → Stop), respectively. A fifth patient was shown to be homozygous for a frameshift mutation in plasminogen exon 14 (Gly565ins-G). In two unrelated subjects with ligneous conjunctivitis no mutations in the plasminogen gene were identified. Our results suggest that the Lys19 → Glu mutation is the most prevalent mutation in the plasminogen gene of patients with ligneous conjunctivitis.


2001 ◽  
Vol 32 (2) ◽  
pp. 179-183 ◽  
Author(s):  
Uǧur Özçelik ◽  
Zuhal Akçören ◽  
Deniz Anadol ◽  
Nural Kiper ◽  
Mehmet Orhon ◽  
...  

Blood Reviews ◽  
2007 ◽  
Vol 21 ◽  
pp. S104
Author(s):  
E. Maka ◽  
G. Veres ◽  
J. Bókay ◽  
P. Domsa ◽  
O. Lukáts

1998 ◽  
Vol 24 (06) ◽  
pp. 605-612 ◽  
Author(s):  
Anne-Marie Mingers ◽  
Anton Philapitsch ◽  
Hans Schwarz ◽  
Petra Zeitler ◽  
Hans Kreth

2005 ◽  
Vol 36 (02) ◽  
pp. 108-111 ◽  
Author(s):  
A. Aslan ◽  
U. Ozcelik ◽  
D. Dogru ◽  
A. Olmez ◽  
G. Turanlı ◽  
...  

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