Ligneous conjunctivitis associated with type I plasminogen deficiency: A rare case

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

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.


Author(s):  
Park Benjamin J ◽  
Camoriano David ◽  
Vital Mark C ◽  
Chévez-Barrios Patricia ◽  
Goosey John D ◽  
...  

Purpose: To describe management of a patient with ligneous conjunctivitis secondary to plasminogen deficiency and review the literature on treatment with plasminogen. Design: Interventional case report. Methods: A 6-year-old Caucasian girl developed ligneous conjunctivitis recalcitrant to debridement and treatment with topical cyclosporin A and steroids. The literature was reviewed regarding treatment of ligneous conjunctivitis with plasminogen. Results: The patient was treated with plasminogen containing drops derived from her father’s fresh frozen plasma. The ligneous conjunctivitis resolved, and the patient was managed on these drops for the following 8 years, initiating treatment for flares and tapering according to clinical response. Conclusions: Plasminogen drops concentrated from fresh frozen plasma are a safe and effective option for long-term treatment of ligneous conjunctivitis.


Author(s):  
Patrick Watts ◽  
Shahab H. Agha ◽  
Maha Mameesh ◽  
Phillip Conor ◽  
Anuradha Ganesh ◽  
...  

2006 ◽  
Vol 26 (S 02) ◽  
pp. S3-S14 ◽  
Author(s):  
P. Innerhofer

SummaryGuidelines of official societies for diagnosis and therapy of intraoperatively occurring hypocoagulability rely mainly on data of patients receiving whole blood transfusions. They recommend -provided that laboratory evaluation shows deficiency (values >1.5 fold normal)- administration of fresh frozen plasma, cryoprecipitate and platelet concentrates (platelet count <50 000 or <100 000/μl). This article describes the pathogenesis of coagulopathy in the light of the special intraoperative setting, emphasizes recent changes of blood component preparation, transfusion triggers, effects of volume therapy and challenges standard laboratory assays as reliable guide for intraoperative hemostatic therapy. The role of thrombelastographic monitoring is discussed as well as an alternative strategy to compensate deficiencies by the use of coagulation factor concentrates instead of or in addition to transfusion of FFP, a new concept which is illustrated by the presentation of an actual case report.


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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