Ligneous conjunctivitis in a patient with Crohn’s disease

Ocular Immunology and Uveitis Unit, Institute of Ophthalmobiology (IOBA), University of Valladolid, Valladolid, Spain and Hospital Universitario de Valladolid, Spain.
Clinical ophthalmology (Auckland, N.Z.) 04/2008; 2(1):203-6.
Source: PubMed


Report the case of a patient with Crohn's disease and ligneous conjunctivitis.
Interventional case report of a 27-year old female patient, with a 4-year history of Crohn's disease, was seen for right eye conjunctival ulcer after resolved chemical trauma. Conjunctival biopsy was performed for histopathology, immunofluorescence microscopy, and immunohistochemistry testing.
Microbiology tests were negative for bacteria and fungi. PCR was negative for atypical mycobacteria and Herpes. Ocular Crohn's disease, Whipple disease, and amyloidosis were ruled out. Immunofluorescence microscopy revealed characteristic IgG kappa and lambda light chain deposits, and fibrin deposition was confirmed through Lendrum's Martius, Scarlet, and Blue technique. Endogenous plasminogen levels were normal. Recurrent ulcers did not resolve after treatment with infliximab, but only after four surgeries, topical steroids, 1% cyclosporine, heparin (5000 units/ml), and hyaluronidase (1.5 mg/ml). After 9 months of follow-up, nasal symblepharon was observed as sequela.
We report the case of a patient with unilateral ligneous conjunctivitis, triggered by chemical injury, and Crohn's disease.

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Available from: María Carmen Méndez, Jun 19, 2014
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    • "It is possible that plasminogen may be only one factor involved in the clinical syndrome and that the identified PLG mutation is not enough alone to cause clinical signs of LM. This also suggests the potential for other abnormalities in proteins in the fibrinolytic pathway and/or that an environmental factor is involved in development of the condition (Fuentes-P aez et al. 2008). Given the possibility of a truncated plasminogen protein that lacks proteolytic activity in affected individuals, it is possible that other proteins are able to provide compensating functions which may also be abnormal in affected patients. "
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    ABSTRACT: Ligneous membranitis (LM) is a rare chronic inflammatory condition of the mucous membranes associated with plasminogen (encoded by PLG) deficiency in affected humans and dogs. In human, the condition is genetic in nature with numerous mutations and polymorphisms in PLG identified in affected individuals and related family members. The condition is uncommonly reported in dogs and, to date, no genetic studies have been performed. We identified related Scottish Terriers (littermates) with severe LM and unaffected relatives (sire, dam and a sibling from a previous litter). Plasma plasminogen activity was below normal in one affected dog but within normal reference intervals for the other. Sequencing of PLG from the affected dogs revealed a homozygous A>T single nucleotide polymorphism in an intron donor site (c.1256+2T>A). The related, unaffected dogs displayed heterozygous alleles at this position (c.1256+2T/A), whereas no mutation was detected in unaffected, non-related control dogs. This is the first report to identify gene polymorphisms associated with LM in dogs.
    Animal Genetics 09/2015; 46(6). DOI:10.1111/age.12339 · 2.21 Impact Factor