MCID: LGN005
MIFTS: 40

Ligneous Conjunctivitis

Categories: Eye diseases, Rare diseases, Reproductive diseases

Aliases & Classifications for Ligneous Conjunctivitis

MalaCards integrated aliases for Ligneous Conjunctivitis:

Name: Ligneous Conjunctivitis 52 71
Conjunctivitis, Ligneous 54
Conjunctivitis Lignosa 52

Classifications:



Summaries for Ligneous Conjunctivitis

NIH Rare Diseases : 52 Ligneous conjunctivitis is a rare disorder characterized by the buildup of a protein called fibrin which causes inflammation of the conjunctiva (conjunctivitis) and leads to thick, woody (ligneous), inflamed growths that are yellow, white, or red. Ligneous conjunctivitis most often occurs on the inside of the eyelids, but may also affect the sclera, cornea and pupil, leading to vision loss. A systemic form of the condition may occur, affecting the mucous membranes of the larynx, vocal chords, nose, trachea, bronchi, vagina, cervix, and gingiva. The cause of ligneous conjunctivitis is unknown. Autosomal recessive inheritance has been suggested in some cases. Ligneous conjunctivitis is sometimes associated with a condition known as congenital plasminogen deficiency .

MalaCards based summary : Ligneous Conjunctivitis, also known as conjunctivitis, ligneous, is related to obstructive hydrocephalus and gingivitis. An important gene associated with Ligneous Conjunctivitis is PLG (Plasminogen), and among its related pathways/superpathways are Degradation of the extracellular matrix and Transcriptional misregulation in cancer. The drugs Fibrinolytic Agents and Plasminogen have been mentioned in the context of this disorder. Affiliated tissues include kidney, cervix and trachea, and related phenotypes are macrocephaly and global developmental delay

Wikipedia : 74 Ligneous conjunctivitis is a rare form of chronic conjunctivitis characterized by recurrent, fibrin-rich... more...

Related Diseases for Ligneous Conjunctivitis

Diseases related to Ligneous Conjunctivitis via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 96)
# Related Disease Score Top Affiliating Genes
1 obstructive hydrocephalus 30.0 PLG PLAT
2 gingivitis 29.8 PLAT ELANE
3 plasminogen deficiency, type i 12.6
4 congenital plasminogen deficiency 12.0
5 conjunctivitis 11.2
6 hydrocephalus 10.6
7 pseudomembranous conjunctivitis 10.5
8 congenital hydrocephalus 10.4
9 chronic conjunctivitis 10.4
10 vasculitis 10.4
11 periodontitis 10.3
12 otitis media 10.2
13 strabismus 10.2
14 autosomal recessive disease 10.2
15 ptosis 10.2
16 mechanical strabismus 10.2
17 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
18 conjunctival disease 10.2
19 dandy-walker syndrome 10.1
20 factor xii deficiency 10.1
21 hydrocephalus due to congenital stenosis of aqueduct of sylvius 10.1
22 yemenite deaf-blind hypopigmentation syndrome 10.1
23 branchiootic syndrome 1 10.1
24 ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitus 10.1
25 igg4-related disease 10.1
26 monocular esotropia 10.1
27 gastric ulcer 10.1
28 respiratory failure 10.1
29 entropion 10.1
30 von willebrand's disease 10.1
31 bronchiolitis 10.1
32 cellulitis 10.1
33 kidney disease 10.1
34 peptic ulcer disease 10.1
35 cataract 10.1
36 whipple disease 10.1
37 crohn's disease 10.1
38 amyloidosis 10.1
39 keratoconjunctivitis 10.1
40 esotropia 10.1
41 hydrocele 10.1
42 vernal keratoconjunctivitis 10.1
43 posttransplant acute limbic encephalitis 10.1
44 basal ganglia cerebrovascular disease 10.0 PLG PLAT
45 anterior cerebral artery infarction 10.0 PLG PLAT
46 basilar artery occlusion 10.0 PLG PLAT
47 acute cor pulmonale 10.0 PLG PLAT
48 acute pulmonary heart disease 10.0 PLG PLAT
49 marantic endocarditis 10.0 PLG PLAT
50 brain stem infarction 10.0 PLG PLAT

Graphical network of the top 20 diseases related to Ligneous Conjunctivitis:



Diseases related to Ligneous Conjunctivitis

Symptoms & Phenotypes for Ligneous Conjunctivitis

Human phenotypes related to Ligneous Conjunctivitis:

31 (show all 31)
# Description HPO Frequency HPO Source Accession
1 macrocephaly 31 hallmark (90%) HP:0000256
2 global developmental delay 31 hallmark (90%) HP:0001263
3 cerebellar hypoplasia 31 hallmark (90%) HP:0001321
4 poor wound healing 31 hallmark (90%) HP:0001058
5 chronic irritative conjunctivitis 31 hallmark (90%) HP:0007717
6 epiphora 31 hallmark (90%) HP:0009926
7 gingival overgrowth 31 frequent (33%) HP:0000212
8 gingivitis 31 frequent (33%) HP:0000230
9 recurrent otitis media 31 frequent (33%) HP:0000403
10 gastrointestinal inflammation 31 frequent (33%) HP:0004386
11 periodontitis 31 frequent (33%) HP:0000704
12 recurrent pharyngitis 31 frequent (33%) HP:0100776
13 recurrent pneumonia 31 frequent (33%) HP:0006532
14 premature loss of teeth 31 frequent (33%) HP:0006480
15 abnormality of the larynx 31 frequent (33%) HP:0001600
16 keratoconjunctivitis 31 frequent (33%) HP:0001096
17 recurrent bronchitis 31 frequent (33%) HP:0002837
18 stomatitis 31 frequent (33%) HP:0010280
19 hyperreflexia 31 occasional (7.5%) HP:0001347
20 hydrocephalus 31 occasional (7.5%) HP:0000238
21 blindness 31 occasional (7.5%) HP:0000618
22 abnormality of the gallbladder 31 occasional (7.5%) HP:0005264
23 papule 31 occasional (7.5%) HP:0200034
24 dandy-walker malformation 31 occasional (7.5%) HP:0001305
25 nephrolithiasis 31 occasional (7.5%) HP:0000787
26 abnormality of the mediastinum 31 occasional (7.5%) HP:0045026
27 abnormality of fontanelles 31 occasional (7.5%) HP:0011328
28 nephritis 31 occasional (7.5%) HP:0000123
29 duodenal ulcer 31 occasional (7.5%) HP:0002588
30 vaginitis 31 occasional (7.5%) HP:0030683
31 cervicitis 31 occasional (7.5%) HP:0030160

Drugs & Therapeutics for Ligneous Conjunctivitis

Drugs for Ligneous Conjunctivitis (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 Fibrinolytic Agents Phase 2, Phase 3
2 Plasminogen Phase 2, Phase 3
3 Pharmaceutical Solutions Phase 2, Phase 3
4 Ophthalmic Solutions Phase 2, Phase 3

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Historically Controlled Phase II/III Study to Evaluate Efficacy and Safety of Kedrion Human Plasminogen Eye Drop Preparation in Patients Diagnosed With Ligneous Conjunctivitis Active, not recruiting NCT01554956 Phase 2, Phase 3

Search NIH Clinical Center for Ligneous Conjunctivitis

Genetic Tests for Ligneous Conjunctivitis

Anatomical Context for Ligneous Conjunctivitis

MalaCards organs/tissues related to Ligneous Conjunctivitis:

40
Kidney, Cervix, Trachea, Eye, Heart, T Cells, Brain

Publications for Ligneous Conjunctivitis

Articles related to Ligneous Conjunctivitis:

(show top 50) (show all 176)
# Title Authors PMID Year
1
Ligneous conjunctivitis: a clinicopathological, immunohistochemical, and genetic study including the treatment of two sisters with multiorgan involvement. 54 61
17701212 2007
2
Polymorphonuclear elastase in patients with homozygous type I plasminogen deficiency and ligneous conjunctivitis. 54 61
10066157 1998
3
An international registry of patients with plasminogen deficiency (HISTORY). 61
32001536 2020
4
[Ligneous conjunctivitis: A diagnosis to consider in the case of a lingering pseudomembranous conjunctivitis!] 61
31262596 2019
5
Prophylactic protocol for dental care in ligneous gingivitis due to severe plasminogen deficiency: Case report and review of literature. 61
30994256 2019
6
Diagnosis of a case of ligneous gingivitis in a patient with moderate plasminogen deficiency. 61
30513391 2019
7
Treatment of ligneous conjunctivitis with heterologous serum. 61
31215592 2019
8
Fresh frozen plasma (Octaplas) and topical heparin in the management of ligneous conjunctivitis. 61
30165199 2019
9
Ligneous conjunctivitis in a Dandy-Walker syndrome: A rare case report. 61
30574926 2019
10
Retraction: Treatment of Ligneous Conjunctivitis Using Topical Plasminogen Therapy in An 8-Week-Old Female Infant. 61
30673103 2019
11
Ligneous conjunctivitis due to plasminogen deficit: Diagnostic and therapeutic approach. With literature review. 61
30442487 2018
12
Amniotic membrane transplantation for ligneous conjunctivitis in a doberman. 61
29482261 2018
13
Clinical, histopathological, and genetic aspects in one case of ligneous conjunctivitis. 61
30058940 2018
14
Treatment of Ligneous Conjunctivitis Using Topical Plasminogen Therapy in an 8-Week-Old Female Infant. 61
30180242 2018
15
Ligneous periodontitis in a child with plasminogen deficiency. 61
29379003 2017
16
Manifestation of tranexamic acid toxicity in chronic kidney disease and kidney transplant patients: A report of four cases and review of literature. 61
26970218 2017
17
Topical Plasminogen as Adjunctive Treatment in Recurrent Ligneous Conjunctivitis. 61
27065432 2017
18
Plasminogen deficiency. 61
27629020 2017
19
Letter 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. 61
26270466 2017
20
Molecular pathogenesis of plasminogen Hakodate: the second Japanese family case of severe type I plasminogen deficiency manifested late-onset multi-organic chronic pseudomembranous mucositis. 61
27193180 2016
21
Co-existing ligneous conjunctivitis and IgG4-related disease. 61
27609168 2016
22
Presentation, clinical pathological and post-mortem findings in three related Scottish terriers with ligneous membranitis. 61
26840763 2016
23
Features and outcome of a glomerulonephropathy associated with ligneous conjunctivitis in a Doberman pinscher dog. 61
27152037 2016
24
[Ligneous conjunctivitis]. 61
26142234 2016
25
Novel plasminogen and hyaluronate sodium eye drop formulation for a patient with ligneous conjunctivitis. 61
27045067 2016
26
Beyond hemostasis: the challenge of treating plasminogen deficiency. A report of three cases. 61
26036227 2016
27
Clinical and histopathological aspects in two cases of ligneous conjunctivitis. 61
27516042 2016
28
Adult-Onset Ligneous Conjunctivitis with Detection of a Novel Plasminogen Gene Mutation and Anti-Plasminogen IgA Antibody: A Clinicopathologic Study and Review of Literature. 61
25674820 2016
29
Successful treatment of ligneous conjunctivitis with topical fresh frozen plasma in an infant. 61
26466233 2015
30
Ligneous conjunctivitis in a patient of juvenile colloid milia: A rare association. 61
26044480 2015
31
A puzzling case: hypoplasminogenemia, ligneous conjunctivitis and hydrocephalus. 61
25006691 2015
32
Root dentin anomaly and a PLG mutation. 61
25281489 2014
33
Tranexamic acid-induced ligneous conjunctivitis with renal failure showed reversible hypoplasminogenaemia. 61
24842363 2014
34
Is the B6.129P2-Plg transgenic mouse an adequate treatment model for patients with ligneous conjunctivitis? 61
24056190 2013
35
Conjunctival instillation of plasminogen eliminates ocular lesion in B6.129P2-Plg(tm1Jld) transgenic mice, a model of ligneous conjunctivitis. 61
23684723 2013
36
Ligneous conjunctivitis in a young patient with homozygous K19E plasminogen gene mutation: a diagnostic and therapeutic challenge. 61
23629776 2013
37
[Ligneous conjunctivitis]. 61
22975272 2012
38
Treatment of ligneous conjunctivitis with amniotic membrane transplantation and topical cyclosporine. 61
23202401 2012
39
Topical fresh frozen plasma and heparin treatment of ligneous conjunctivitis in a Canadian hospital setting. 61
23036559 2012
40
Destructive membranous periodontal disease (ligneous gingivitis): a literature review. 61
21859319 2012
41
Management of ligneous conjunctivitis in a child with plasminogen deficiency. 61
21625933 2011
42
Heparin in the long-term management of ligneous conjunctivitis: a case report and review of literature. 61
21885957 2011
43
Identification of three novel plasminogen (PLG) gene mutations in a series of 23 patients with low PLG activity. 61
21174000 2011
44
Induction of bilateral ligneous conjunctivitis with the use of a prosthetic eye. 61
20524141 2011
45
Ligneous periodontitis is an analog lesion of ligneous conjunctivitis and described 150 years later. 61
19782618 2009
46
Successful treatment of ligneous conjunctivitis with topical cyclosporine and heparin. 61
19840736 2009
47
Ligneous conjunctivitis with oral mucous membrane involvement and decreased plasminogen level. 61
19689523 2009
48
Ligneous conjunctivitis in a plasminogen-deficient dog: clinical management and 2-year follow-up. 61
19604341 2009
49
The first two Japanese cases of severe type I congenital plasminogen deficiency with ligneous conjunctivitis: successful treatment with direct thrombin inhibitor and fresh plasma. 61
19373890 2009
50
Ligneous conjunctivitis with plasminogen deficiency. 61
19006564 2009

Variations for Ligneous Conjunctivitis

Expression for Ligneous Conjunctivitis

Search GEO for disease gene expression data for Ligneous Conjunctivitis.

Pathways for Ligneous Conjunctivitis

GO Terms for Ligneous Conjunctivitis

Cellular components related to Ligneous Conjunctivitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.71 TTR PLG PLAT ELANE
2 extracellular exosome GO:0070062 9.46 TTR PLG PLAT ELANE
3 secretory granule GO:0030141 9.43 PLAT ELANE
4 cell surface GO:0009986 9.43 PLG PLAT ELANE
5 azurophil granule lumen GO:0035578 9.37 TTR ELANE
6 Schaffer collateral - CA1 synapse GO:0098685 9.32 PLG PLAT
7 extracellular space GO:0005615 9.26 TTR PLG PLAT ELANE
8 collagen-containing extracellular matrix GO:0062023 8.8 PLG PLAT ELANE

Biological processes related to Ligneous Conjunctivitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 proteolysis GO:0006508 9.43 PLG PLAT ELANE
2 blood coagulation GO:0007596 9.32 PLG PLAT
3 extracellular matrix disassembly GO:0022617 9.16 PLG ELANE
4 fibrinolysis GO:0042730 8.96 PLG PLAT
5 trans-synaptic signaling by BDNF, modulating synaptic transmission GO:0099183 8.62 PLG PLAT

Molecular functions related to Ligneous Conjunctivitis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 peptidase activity GO:0008233 9.33 PLG PLAT ELANE
2 endopeptidase activity GO:0004175 9.26 PLG ELANE
3 serine-type endopeptidase activity GO:0004252 9.13 PLG PLAT ELANE
4 serine-type peptidase activity GO:0008236 8.8 PLG PLAT ELANE

Sources for Ligneous Conjunctivitis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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