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      Successful treatment of ligneous conjunctivitis with topical fresh frozen plasma in an infant Translated title: Sucesso do tratamento da conjuntivite lenhosa com uso tópico de plasma fresco congelado em uma criança

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          Abstract

          ABSTRACTA 6-month-old female infant presented to our clinic with bilateral eyelid swelling, yellowish-white membranes under both lids, and mucoid ocular discharge. Her aunt had similar ocular problems that were undiagnosed. The conjunctival membranes were excised and histopathological investigation of these membranes showed ligneous conjunctivitis. Further, laboratory examination revealed plasminogen deficiency. A good response was observed to topical fresh frozen plasma (FFP) treatment without systemic therapy, and the membranes did not recur during the treatment. Topical FFP treatment may facilitate rapid rehabilitation and prevent recurrence in patients with ligneous conjunctivitis.

          Translated abstract

          RESUMOUma criança feminina com seis meses de idade se apresentou à nossa clínica com edema palpebral bilateral, membranas brancas amareladas sob as pálpebras de ambos os olhos e descarga mucosa. Sua tia já havia apresentado problemas oculares semelhantes que não foram diagnosticados. As membranas conjuntivais foram excisadas e a investigação histopatológica das membranas demonstraram conjuntivite lenhosa. O diagnóstico de deficiência de plasminogênio foi obtido a partir de um exame laboratorial. Tratamento tópico com plasma fresco congelado (FFP) sem qualquer terapia sistêmica mostrou boa resposta. Não foram observadas recorrências das membranas. O tratamento tópico com FFP pode ajudar a reabilitação rápida e prevenir a recorrência em pacientes com conjuntivite lenhosa.

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          Most cited references10

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          Therapy with a purified plasminogen concentrate in an infant with ligneous conjunctivitis and homozygous plasminogen deficiency.

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            Treatment of ligneous conjunctivitis with topical plasmin and topical plasminogen.

            To describe treatment of a child with recalcitrant ligneous conjunctivitis secondary to a systemic plasminogen deficiency. Interventional case report. A seven-year-old boy developed severe unilateral membranous conjunctivitis recalcitrant to surgical debridement and treatment with topical prednisone, topical cyclosporine, and oral prednisone. Systemic evaluation revealed a severe plasminogen deficiency. Treatment with surgical debridement and topical plasmin was ineffective and resulted in prompt recurrence of dense conjunctival membranes. Treatment with topical plasminogen resulted in dramatic improvement and complete resolution of the membranes. Ligneous conjunctivitis is secondary to a systemic plasminogen deficiency. Treatment with topical plasminogen resulted in prompt resolution of the membranes. Treatment with topical plasmin was ineffective.
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              Ligneous conjunctivitis: a clinicopathological, immunohistochemical, and genetic study including the treatment of two sisters with multiorgan involvement.

              Ligneous conjunctivitis (LC) is a rare disease characterized by wood-like pseudomembranes developing on the ocular and extraocular mucosae secondary to plasminogen (PLG) deficiency. In this paper, we report two cases of LC in two sisters of 57 and 62 years of age that presented with recurrent, bilateral pseudomembranes on conjunctiva and a history of consanguinity and deafness. Pseudomembranes showed superficial and/or subepithelial deposits of eosinophilic amorphous hyaline, amyloid-like material with a variable proportion of granulation tissue, and inflammatory cells. The eosinophilic deposits were negative for Congo red stain, immunoreactive for fibrinogen, and consistently negative for amyloid A component, transthyretin, beta(2)-microglobulin, albumin, fibronectin, collagen type IV, vimentin, and cytokeratins. Among inflammatory cells, a percentage of positivity of roughly 60% for lymphocytes T (CD3+) and 40% for lymphocytes B (CD8+), with a relation of cytotoxic/helper (CD8/4) T cells of 3:2, was found. In one case, nasal polyps and recurrent gastric peptic ulcer were also characterized by the same subepithelial hyaline deposits. A novel homozygous point mutation c.1856 C>T was found in exon 15 of the PLG gene in both patients. Amniotic membrane transplantation was done in one case with promising results.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                abo
                Arquivos Brasileiros de Oftalmologia
                Arq. Bras. Oftalmol.
                Conselho Brasileiro de Oftalmologia (São Paulo )
                1678-2925
                October 2015
                : 78
                : 5
                : 318-319
                Affiliations
                [1 ] TOBB University of Economics and Technology Turkey
                [2 ] TOBB University of Economics and Technology Turkey
                [3 ] TOBB University of Economics and Technology Turkey
                Article
                S0004-27492015000550083
                10.5935/0004-2749.20150083
                26466233
                426ada94-643b-40e9-81a6-3d2eac8f42be

                http://creativecommons.org/licenses/by/4.0/

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                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=0004-2749&lng=en
                Categories
                OPHTHALMOLOGY

                Ophthalmology & Optometry
                Plasminogen deficiency,Fresh frozen plasma,Conjuntivite/terapia,Plasminogênio/análise,Administração tópica,Relatos de casos,Ligneous conjunctivitis

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