Klin Monbl Augenheilkd 2013; 230(4): 346-348
DOI: 10.1055/s-0032-1328375
Der interessante Fall
Georg Thieme Verlag KG Stuttgart · New York

Ligneous Conjunctivitis in a Young Patient with Homozygous K19E Plasminogen Gene Mutation: a Diagnostic and Therapeutic Challenge

Conjunctivitis lignosa bei einer jungen Patientin mit homozygoter K19E-Mutation im Plasminogen-Gen: eine diagnostische und therapeutische Herausforderung
A. Kuonen
1   Jules Gonin Eye Hospital and University of Lausanne, Department of Ophthalmology, Lausanne, Switzerland (Chairman: Prof. L. Zografos)
,
J. Vaudaux
1   Jules Gonin Eye Hospital and University of Lausanne, Department of Ophthalmology, Lausanne, Switzerland (Chairman: Prof. L. Zografos)
,
M. Hamedani
1   Jules Gonin Eye Hospital and University of Lausanne, Department of Ophthalmology, Lausanne, Switzerland (Chairman: Prof. L. Zografos)
,
V. Schuster
4   Universitätsklinik und Poliklinik für Kinder und Jugendliche, Leipzig, Germany (Chairman: Prof. Dr. Wieland Kiess)
,
M. Albisetti
2   Division of Hematology, University Childrenʼs Hospital, Zürich, Switzerland (Chairman: Dr. Dieter von Schulthess)
,
N. von der Weid
3   Division of Hematology and Oncology, University Childrenʼs Hospital UKBB, Basel, Switzerland (Chairman: Prof. Urs Frey)
,
P.-F. Kaeser
1   Jules Gonin Eye Hospital and University of Lausanne, Department of Ophthalmology, Lausanne, Switzerland (Chairman: Prof. L. Zografos)
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Publikationsverlauf

Publikationsdatum:
29. April 2013 (online)

Background

Ligneous conjunctivitis is a chronic disease that may be triggered by ocular trauma or surgery in patients with type I plasminogen deficiency [1], [2]. In a recent review of the literature, the prevalence of type-I plasminogen deficiency is only 16 cases per 1 000 000 [1]. The hallmark of ligneous conjunctivitis is the presence of fibrin-rich pseudomembraneous lesions on tarsal conjunctivae.

 
  • References

  • 1 Schuster V, Seregard S. Ligneous conjunctivitis. Surv Ophthalmol 2003; 48: 369-388
  • 2 Schuster V, Hügle B, Tefs K. Plasminogen deficiency. J Thromb Haemost 2007; 5: 2315-2322
  • 3 Tefs K, Gueorguieva M, Klammt J et al. Molecular and clinical spectrum of type I plasminogen deficiency: a series of 50 patients. Blood 2006; 108: 3021-3026
  • 4 Klammt J, Kobelt L, Aktas D et al. Identification of three novel plasminogen (PLG) gene mutations in a series of 23 patients with low PLG activity. Thromb Haemost 2011; 105: 454-460
  • 5 Gürlü VP, Demir M, Alimgil ML et al. Systemic and topical fresh-frozen plasma treatment in a newborn with ligneous conjunctivitis. Cornea 2008; 27: 501-503
  • 6 Schott D, Dempfle CE, Beck P et al. Therapy with a purified plasminogen concentrate in an infant with ligneous conjunctivitis and homozygous plasminogen deficiency. N Engl J Med 1998; 339: 1679-1686
  • 7 Caputo R, Pucci N, Mori F et al. Long-term efficacy of surgical removal of pseudomembranes in a child with ligneous conjunctivitis treated with plasminogen eyedrops. Thromb Haemost 2008; 100: 1196-1198
  • 8 Hiremath M, Helder J, Newall F et al. Heparin in the long-term management of ligneous conjunctivitis: a case report and review of literature. Blood Coag Fibrinolysis 2011; 22: 606-609
  • 9 De Cock R, Ficker LA, Dart JG et al. Topical heparin in the treatment of ligneous conjunctivitis. Ophthalmology 1995; 102: 1654-1659
  • 10 Azad N, Zafar S, Khan A. Successful treatment of ligneous conjunctivitis with topical cyclosporine and heparin. JAAPOS 2009; 13: 519-520