Doença multissistêmica rara caracterizada por fibrinólise extracelular marcadamente prejudicada, levando à formação de pseudomembranas lenhosas (ricas em fibrina) nas mucosas.
Introdução
O que você precisa saber de cara
Doença multissistêmica rara caracterizada por fibrinólise extracelular marcadamente prejudicada, levando à formação de pseudomembranas lenhosas (ricas em fibrina) nas mucosas.
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 13 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 28 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição.
Plasmin dissolves the fibrin of blood clots and acts as a proteolytic factor in a variety of other processes including embryonic development, tissue remodeling, tumor invasion, and inflammation. In ovulation, weakens the walls of the Graafian follicle. It activates the urokinase-type plasminogen activator, collagenases and several complement zymogens, such as C1, C4 and C5 (PubMed:6447255). Cleavage of fibronectin and laminin leads to cell detachment and apoptosis. Also cleaves fibrin, thrombosp
Secreted
Plasminogen deficiency
A disorder characterized by decreased serum plasminogen activity. Two forms of the disorder are distinguished: type 1 deficiency is additionally characterized by decreased plasminogen antigen levels and clinical symptoms, whereas type 2 deficiency, also known as dysplasminogenemia, is characterized by normal, or slightly reduced antigen levels, and absence of clinical manifestations. Plasminogen deficiency type 1 results in markedly impaired extracellular fibrinolysis and chronic mucosal pseudomembranous lesions due to subepithelial fibrin deposition and inflammation. The most common clinical manifestation of type 1 deficiency is ligneous conjunctivitis in which pseudomembranes formation on the palpebral surfaces of the eye progresses to white, yellow-white, or red thick masses with a wood-like consistency that replace the normal mucosa.
Medicamentos e terapias
Mecanismo: Fibrinogen hydrolytic enzyme
Variantes genéticas (ClinVar)
78 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
6 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Conjuntivite lenhosa
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
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Outros ensaios clínicos
4 ensaios clínicos encontrados, 1 ativos.
Publicações mais relevantes
Clinical evidence on allogeneic blood-derived eye drops in ocular surface and corneal diseases.
To systematically review clinical studies on allogeneic blood-derived eye drops in ocular surface and corneal diseases. A PRISMA-guided search of PubMed, Google Scholar, Scopus, Web of Science, and Embase (1985-September 2025) identified eligible clinical studies on allogeneic eye drops for ocular surface and corneal diseases. Two reviewers independently extracted data and assessed study quality using Joanna Briggs Institute tools. Sixty studies (1733 participants; 2374 eyes) met inclusion criteria, with 1423 patients (1738 eyes) receiving allogeneic eye drops. The cohort included 929 females (53.6%), 562 males (32.4%), and 242 unspecified (14.0%), with a mean age of 42.3 ± 21.8 years. Indications were dry eye disease (30.0%), mixed ocular surface disorders (21.7%), ligneous conjunctivitis (20.0%), persistent epithelial defects, erosions, and ulcers (18.3%), neurotrophic keratopathy (5.0%), and chemical or toxin-related injuries (5.0%). Treatments comprised allogeneic serum (47.4%), umbilical cord serum (43.2%), cord blood platelet lysate (10.1%), platelet-poor plasma/fresh frozen plasma (6.0%), plasma rich in growth factors (2.1%), and platelet-rich plasma (0.5%). Across 1738 treated eyes, reported outcomes were generally consistent, with improvements observed in patient-reported symptoms, epithelial healing, visual outcomes, and adverse events reported in <2% of cases. In dry eye disease, the largest relative improvements were reported for symptom scores (28.3%) and epithelial healing (24.9%), with smaller gains in tear-based measures (14-17%). Mixed ocular surface disorders showed symptom relief (28.5%), epithelial healing (16.9%), and BCVA improvement (7.7%). Ligneous conjunctivitis achieved complete/near remission in 1.5%, persistent epithelial defects healed in 10.2% with 0.5% recurrence, neurotrophic keratopathy showed 2.1% resolution with improved sensitivity, and chemical burns achieved 2.0% complete closure with better clarity and vision. Overall, patient-reported symptoms and epithelial healing emerged as the most consistently responsive outcomes, while reported safety profiles remained favorable. While many studies demonstrated acceptable methodological quality within their respective designs, the overall evidence base was predominantly composed of observational and non-comparative studies, which limits overall certainty. Allogeneic blood-derived eye drops are associated with meaningful clinical improvement across ocular surface diseases and demonstrate favorable short-to mid-term safety. Further controlled studies with standardized protocols, longer follow-up, and cost-effectiveness analyses are needed.
Diagnostically challenging ligneous conjunctivitis with confirmed PLG variants: clinical and genetic insights.
This study reports the ocular and systemic manifestations, genetic findings, and management approaches in 10 patients diagnosed with ligneous conjunctivitis (LC) and followed at a university hospital. In this retrospective case series, medical records were retrospectively reviewed to collect demographic characteristics, age at diagnosis, family history, serum plasminogen (PLG) activity levels, ocular/systemic findings, and treatment modalities. Ten patients (7 females, 3 males; age range: 2-40 years) were followed for a mean duration of 9.4 ± 5.5 years. PLG activity was markedly reduced (18-25%) in six individuals. Systemic comorbidities included hydrocephalus (n = 3), gingivitis (n = 3), cervicitis/vaginitis (n = 4), menstrual irregularities (n = 2), infertility (n = 2), dacryocystitis (n = 2), epilepsy (n = 1), growth retardation (n = 1), deafness (n = 1), and brain tumor (n = 1). Whole-exome sequencing identified four distinct PLG variants, including homozygous pathogenic variants in five patients and a heterozygous variant in one. Treatment strategies involved pseudomembrane excision, topical heparin, corticosteroids, cyclosporine, and fresh frozen plasma (FFP). Systemic FFP was administered in selected cases. Additional procedures included amniotic membrane transplantation (n = 4) and cataract surgery (n = 3). The diagnosis of LC is based on the integration of clinical and genetic findings, characterized by recurrent, firm pseudomembranes on the tarsal conjunctiva and often supported by a positive family history or parental consanguinity. Reduced PLG activity, histopathological confirmation of fibrin-rich membranes, and supportive genetic findings further substantiate the diagnosis.
Blood banks saving sight: collaboration for successful treatment of ligneous conjunctivitis.
The purpose of this study was to report the presentation and outcomes of three cases of ligneous conjunctivitis (LC) treated successfully with topical plasminogen concentrate (TPC). Aggregate results of three n-of-1 clinical trials. Three patients from a tertiary pediatric health care center with a recent diagnosis of LC due to plasminogen deficiency. Three patients with LC and low serum plasminogen activity were enrolled in n = 1 clinical trials. Patients received hourly TPC eye drops in the affected eye, tapered per treatment response to once daily. The first patient presented with Streptococcus pyogenes corneal ulcer and pseudomembranes (PMs) in his right eye following an upper respiratory tract infection. Despite aggressive topical antibiotics and subsequent addition of topical corticosteroids, corneal perforation occurred. Urgent penetrating keratoplasty, peeling of the PMs, and amniotic membrane transplantation were performed to both upper and lower palpebral conjunctivas. PMs recurred rapidly, prompting further investigation and the diagnosis of LC. The patient was started on hourly TPC and improved promptly, with complete resolution of PMs after 4 weeks. The second and third patients were started on the TPC protocol shortly after presentation. They showed resolution of PMs after 3 weeks and 1 week, respectively. Two patients were stable under maintenance TPC once daily at last follow-up with no recurrences. One patient had PM recurrence with upper respiratory tract infections, requiring intermittent increases of TPC to hourly dosage. Despite regulatory barriers, TPC yielded highly favorable outcomes in these challenging cases of LC.
Treatment of Ligneous Conjunctivitis With Intravenous Plasminogen.
This case report discusses the diagnosis and treatment of ligneous conjunctivitis in a man who presented with a left upper eyelid pseudomembrane of 1-year duration, recalcitrant periodontitis, and chronic sinusitis.
Fresh frozen plasma therapy in type I plasminogen deficiency: a case of ligneous conjunctivitis with hydrocephalus.
Plasminogen deficiency is an ultra-rare autosomal recessive disorder characterized by impaired fibrinolysis and the formation of fibrin-rich pseudomembranes. Ligneous conjunctivitis is the most common manifestation, whereas central nervous system involvement, such as hydrocephalus, is rare. Early recognition is essential to prevent irreversible complications. We report a 3-year-old girl with progressive macrocephaly, antenatal hydrocephalus, and persistent pseudomembranous conjunctivitis. Imaging showed fused lateral ventricles and agenesis of the corpus callosum, consistent with obstructive hydrocephalus requiring multiple ventriculoperitoneal shunt revisions. Genetic testing confirmed type I plasminogen deficiency with a homozygous PLG mutation. Recombinant plasminogen was unavailable, and she was managed with fresh frozen plasma (FFP) infusions plus ophthalmic and anticonvulsant therapy. Regular FFP stabilized her ocular and neurological condition, though recurrent shunt complications necessitated repeated hospitalizations. This case illustrates the diagnostic challenges of type I plasminogen deficiency and highlights FFP as a practical therapeutic option in resource-limited settings.
Publicações recentes
Clinical evidence on allogeneic blood-derived eye drops in ocular surface and corneal diseases.
Fresh frozen plasma therapy in type I plasminogen deficiency: a case of ligneous conjunctivitis with hydrocephalus.
Diagnostically challenging ligneous conjunctivitis with confirmed PLG variants: clinical and genetic insights.
Treatment of Ligneous Conjunctivitis With Intravenous Plasminogen.
Advancing Understanding of Ligneous Conjunctivitis: Bridging Pathogenesis, Diagnosis, and Therapy.
📚 EuropePMC173 artigos no totalmostrando 74
Clinical evidence on allogeneic blood-derived eye drops in ocular surface and corneal diseases.
The ocular surfaceFresh frozen plasma therapy in type I plasminogen deficiency: a case of ligneous conjunctivitis with hydrocephalus.
Oxford medical case reportsDiagnostically challenging ligneous conjunctivitis with confirmed PLG variants: clinical and genetic insights.
Ophthalmic geneticsTreatment of Ligneous Conjunctivitis With Intravenous Plasminogen.
JAMA ophthalmologyAdvancing Understanding of Ligneous Conjunctivitis: Bridging Pathogenesis, Diagnosis, and Therapy.
CorneaCryo-Depleted Plasma Infusions for Ligneous Conjunctivitis.
Case reports in ophthalmologyBlood banks saving sight: collaboration for successful treatment of ligneous conjunctivitis.
Canadian journal of ophthalmology. Journal canadien d'ophtalmologieTreatment with Plasma Transfusion and Plasma-Derived Human Plasminogen in a Newborn with Plasminogen Deficiency and Recurrent Hydrocephalus: A Case Report.
NeonatologyLigneous Conjunctivitis with Plasminogen Deficiency Treated with Topical Allogeneic Serum Drops: A Case Report.
Beyoglu eye journalSafety and Efficacy of Long-Term Treatment of Type 1 Plasminogen Deficient Patients With Intravenous Plasminogen Replacement Therapy.
Haemophilia : the official journal of the World Federation of HemophiliaLigneous Conjunctivitis Treated with Intravenous Human Plasminogen.
OphthalmologyOral mucous membrane tarsal patch grafting: broadening indications and long-term outcomes.
Orbit (Amsterdam, Netherlands)Congenital hydrocephalus and ligneous conjunctivitis in two children with severe type I plasminogen deficiency: A case report and literature review.
SAGE open medical case reportsPlasminogen missense variants and their involvement in cardiovascular and inflammatory disease.
Frontiers in cardiovascular medicineLigneous conjunctivitis: Fresh-frozen plasma and heparin use intra-and postoperatively, a report of two cases.
Arquivos brasileiros de oftalmologiaPersistent Hydrocephalus, Shunt, and Subglottic Stenosis in a Newborn with Plasminogen Deficiency due to Delayed Treatment with Plasminogen Concentrates: A Case Report.
NeonatologyA new treatment changes the game: An innovative management of a child suffering from severe ligneous conjunctivitis with type 1 plasminogen deficiency.
The ocular surfaceAmniotic membrane transplantation: structural and biological properties, tissue preparation, application and clinical indications.
Eye (London, England)Acquired plasminogen deficiency and ligneous conjunctivitis associated with chronic tranexamic acid use.
The Lancet. HaematologyRecurrent Meningitis and Its Rare Association With Ligneous Conjunctivitis and Congenital Plasminogen Deficiency.
CureusPlasminogen Deficiency: A Case Report and Review.
CureusCombined medical and surgical approach in the management of ligneous conjunctivitis in a pediatric patient: A case report.
International journal of surgery case reportsLong-term follow-up in a pediatric patient with Ligneous Conjunctivitis due to PLG gene mutation in topical plasminogen treatment after successful use of ocular prosthesis for aesthetic rehabilitation: a case report.
Italian journal of pediatricsDiagnosis of Immunoglobulin G4-related disease in a child with ligneous conjunctivitis: a novel mutation in plasminogen gene and plasminogen activator inhibitor-1 polymorphism.
Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosisA case of IgG4-positive ligneous conjunctivitis mistaken for a conjunctival mass.
Diagnostic pathologyConjunctivitis: A Primer on Conjunctival Biopsy and Approach to Histopathologic Diagnosis.
Advances in anatomic pathologyDelayed-onset ligneous conjunctivitis as a rare association with congenital hydrocephalous: a case report and review of the literature.
Digital journal of ophthalmology : DJOLigneous conjunctivitis and use of human plasminogen eyedrops: The Italian experience.
Haemophilia : the official journal of the World Federation of HemophiliaLigneous Conjunctivitis Secondary to Viral Conjunctivitis.
CorneaLigneous conjunctivitis mimicking preseptal cellulitis in a 3-month-old infant.
Ophthalmic geneticsTreatment of Ligneous Conjunctivitis with Plasminogen Eyedrops.
OphthalmologyRecurrent ligneous conjunctivitis after cataract surgery in a 67-year-old male patient: a case report.
BMC ophthalmologyThe first Moroccan case of severe type II congenital plasminogen deficiency with ligneous conjunctivitis.
Annales de biologie cliniqueLigneous conjunctivitis exacerbated by Pseudomonas aeruginosa - Diagnostic and therapeutic approach.
Oman journal of ophthalmologyUse of topical allogenic fresh-frozen plasma drops in the treatment of ligneous conjunctivitis.
Canadian journal of ophthalmology. Journal canadien d'ophtalmologieA large deletion in the Plasminogen gene is associated with ligneous membranitis in a Maltese dog.
Animal geneticsLigneous conjunctivitis associated with type I plasminogen deficiency: A rare case.
European journal of ophthalmology[Pharmaceutical supply of human plasminogen replacement therapy for the treatment of ligneous conjunctivitis: Logistics for a university hospital].
Journal francais d'ophtalmologieA Novel mRNA Modification Mutation in a Patient With Ligneous Conjunctivitis Coexisting With Heterozygous Familial Mediterranean Fever Mutation.
CorneaNovel homozygous mutation of plasminogen in ligneous conjunctivitis: a case report and literature review.
Ophthalmic geneticsTreatment of ligneous conjunctivitis with fresh frozen plasm in twin babies.
Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and StrabismusAnterior Segment Surgeries Under Topical Fresh Frozen Plasma Treatment in Ligneous Conjunctivitis.
CorneaLigneous conjunctivitis masquerading as a large, recurrent conjunctival tumor.
Journal francais d'ophtalmologieRepurposing of Plasminogen: An Orphan Medicinal Product Suitable for SARS-CoV-2 Inhalable Therapeutics.
Pharmaceuticals (Basel, Switzerland)Functional Fibrinolysis Assays Reveal Different Mechanisms underlying Plasminogen Dysfunction in Ligneous Conjunctivitis.
Thrombosis and haemostasisLigneous Periodontitis in a Patient with Type 1 Plasminogen Deficiency: A Case Report and Review of the Literature.
Case reports in dentistryAn international registry of patients with plasminogen deficiency (HISTORY).
Haematologica[Ligneous conjunctivitis: A diagnosis to consider in the case of a lingering pseudomembranous conjunctivitis!].
Journal francais d'ophtalmologieTreatment of ligneous conjunctivitis with heterologous serum.
Einstein (Sao Paulo, Brazil)Prophylactic protocol for dental care in ligneous gingivitis due to severe plasminogen deficiency: Case report and review of literature.
Haemophilia : the official journal of the World Federation of HemophiliaCongenital plasminogen deficiency with long standing pseudomembranous conjunctival and genital lesions.
JAAD case reportsLigneous conjunctivitis in a Dandy-Walker syndrome: A rare case report.
Indian journal of ophthalmologyDiagnosis of a case of ligneous gingivitis in a patient with moderate plasminogen deficiency.
Journal of stomatology, oral and maxillofacial surgeryLigneous conjunctivitis due to plasminogen deficit: Diagnostic and therapeutic approach. With literature review.
Journal francais d'ophtalmologieTreatment of Ligneous Conjunctivitis Using Topical Plasminogen Therapy in an 8-Week-Old Female Infant.
Journal of pediatric ophthalmology and strabismusFresh frozen plasma (Octaplas) and topical heparin in the management of ligneous conjunctivitis.
Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and StrabismusClinical, histopathological, and genetic aspects in one case of ligneous conjunctivitis.
Ophthalmic geneticsAmniotic membrane transplantation for ligneous conjunctivitis in a doberman.
Veterinary ophthalmologyLigneous periodontitis in a child with plasminogen deficiency.
Nigerian journal of clinical practiceTreatment of plasminogen deficiency patients with fresh frozen plasma.
Pediatric blood & cancerCo-existing ligneous conjunctivitis and IgG4-related disease.
Indian journal of ophthalmologyClinical and histopathological aspects in two cases of ligneous conjunctivitis.
Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologieMolecular pathogenesis of plasminogen Hakodate: the second Japanese family case of severe type I plasminogen deficiency manifested late-onset multi-organic chronic pseudomembranous mucositis.
Journal of thrombosis and thrombolysisFeatures and outcome of a glomerulonephropathy associated with ligneous conjunctivitis in a Doberman pinscher dog.
The Canadian veterinary journal = La revue veterinaire canadienneTopical Plasminogen as Adjunctive Treatment in Recurrent Ligneous Conjunctivitis.
Ophthalmic plastic and reconstructive surgeryNovel plasminogen and hyaluronate sodium eye drop formulation for a patient with ligneous conjunctivitis.
American journal of health-system pharmacy : AJHP : official journal of the American Society of Health-System PharmacistsManifestation of tranexamic acid toxicity in chronic kidney disease and kidney transplant patients: A report of four cases and review of literature.
Nephrology (Carlton, Vic.)Presentation, clinical pathological and post-mortem findings in three related Scottish terriers with ligneous membranitis.
The Journal of small animal practiceSuccessful treatment of ligneous conjunctivitis with topical fresh frozen plasma in an infant.
Arquivos brasileiros de oftalmologiaLetter Regarding "Adult-Onset Ligneous Conjunctivitis with Detection of a Novel Plasminogen Gene Mutation and Anti-Plasminogen IgA Antibody: A Clinicopathologic Study and Review of Literature" by Y. Tu et al.
Seminars in ophthalmology[Ligneous conjunctivitis].
Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen GesellschaftLigneous conjunctivitis in a patient of juvenile colloid milia: A rare association.
Indian journal of ophthalmologyBeyond hemostasis: the challenge of treating plasminogen deficiency. A report of three cases.
Journal of thrombosis and thrombolysisAdult-Onset Ligneous Conjunctivitis with Detection of a Novel Plasminogen Gene Mutation and Anti-Plasminogen IgA Antibody: A Clinicopathologic Study and Review of Literature.
Seminars in ophthalmologyAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Clinical evidence on allogeneic blood-derived eye drops in ocular surface and corneal diseases.
- Diagnostically challenging ligneous conjunctivitis with confirmed PLG variants: clinical and genetic insights.
- Blood banks saving sight: collaboration for successful treatment of ligneous conjunctivitis.
- Treatment of Ligneous Conjunctivitis With Intravenous Plasminogen.
- Fresh frozen plasma therapy in type I plasminogen deficiency: a case of ligneous conjunctivitis with hydrocephalus.
- Advancing Understanding of Ligneous Conjunctivitis: Bridging Pathogenesis, Diagnosis, and Therapy.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:97231(Orphanet)
- OMIM OMIM:217090(OMIM)
- MONDO:0009009(MONDO)
- GARD:4380(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Q6546687(Wikidata)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
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