MCID: CCT002
MIFTS: 55

Cicatricial Pemphigoid malady

Rare diseases, Eye diseases, Skin diseases, Immune diseases categories

Summaries for Cicatricial Pemphigoid

About this section
Sources:
44NIH Rare Diseases, 66Wikipedia, 34MalaCards
See all sources

Fully expand this MalaCard

Download this MalaCard
NIH Rare Diseases:44 Cicatricial pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. other mucosal sites that might be affected include the nasopharnyx, larynx, genitalia, rectum, and esophagus. the condition usually begins in late adulthood (e.g. 50's or 60's), affects more women than men, and has a variable prognosis. scarring of the affected  mucosa of the eye may lead to blindness and tends to be the most feared complication. a combination of environmental and genetic factors appear to play a role in the susceptibility of developing benign mucosal pemphigoid. although the specific causes of benign mucosal pemphigoid have not been identified, the condition is considered an autoimmune disease that is characterized by the production of autoantibodies against basement membrane zone antigens such as bp180, bp230, and laminin 5.  treatment is dependent on the person's specific symptoms. last updated: 8/4/2009

MalaCards: Cicatricial Pemphigoid, also known as benign mucous membrane pemphigoid with ocular involvement, is related to ocular cicatricial pemphigoid and bullous pemphigoid. An important gene associated with Cicatricial Pemphigoid is ITGB4 (integrin, beta 4), and among its related pathways are Validated targets of C-MYC transcriptional repression and Non-integrin membrane-ECM interactions. The compounds enterotoxin and allergens have been mentioned in the context of this disorder. Affiliated tissues include skin, eye and testes, and related mouse phenotypes are limbs/digits/tail and digestive/alimentary.

Wikipedia:66 Cicatricial pemphigoid (also known as \"Mucous Membrane Pemphigoid\", \"MMP\", \"Benign mucosal... more...

Aliases & Classifications for Cicatricial Pemphigoid

About this section
Sources:
9Disease Ontology, 44NIH Rare Diseases, 11DISEASES, 63UMLS, 50Orphanet, 46Novoseek, 59SNOMED-CT, 28ICD9CM, 36MeSH, 41NCIt, 27ICD10 via Orphanet, 26ICD10
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Eye diseases, Skin diseases, Immune diseases
Orphanet: 50 
Rare eye diseases


Aliases & Descriptions:

cicatricial pemphigoid 9 44 11
benign mucous membrane pemphigoid with ocular involvement 9 63
ocular pemphigoid 9 50
cicatricial pemphigoid with ocular involvement 9
pemphigoid, benign mucous membrane 63
benign mucous membrance pemphigoid 44
benign mucous membrane pemphigoid 9
benign mucosal pemphigoid 44
pemphigoid cicatricial 46
ocular pemphigus 9


External Ids:

Disease Ontology9 DOID:11656
ICD9CM28 694.6
MeSH36 D010390
NCIt41 C34907
ICD10 via Orphanet27 H13.3*, L12+
ICD1026 H13.3, L12.1

Related Diseases for Cicatricial Pemphigoid

About this section
Sources:
18GeneCards, 19GeneDecks
See all sources

Graphical network of the top 20 diseases related to Cicatricial Pemphigoid:



Diseases related to cicatricial pemphigoid

Symptoms for Cicatricial Pemphigoid

About this section

Drugs & Therapeutics for Cicatricial Pemphigoid

About this section
Sources:
6CenterWatch, 43NIH Clinical Center, 7ClinicalTrials, 63UMLS, 42NDF-RT
See all sources

Approved drugs:

Search CenterWatch for Cicatricial Pemphigoid

Drug clinical trials:

Search ClinicalTrials for Cicatricial Pemphigoid

Search NIH Clinical Center for Cicatricial Pemphigoid

Search CenterWatch for Cicatricial Pemphigoid

Genetic Tests for Cicatricial Pemphigoid

About this section

Anatomical Context for Cicatricial Pemphigoid

About this section
Sources:
34MalaCards
See all sources

MalaCards organs/tissues related to Cicatricial Pemphigoid:

34
Skin, Eye, Testes, Lung, Colon, T cells, Pancreas

Animal Models for Cicatricial Pemphigoid or affiliated genes

About this section
Sources:
38MGI
See all sources

MGI Mouse Phenotypes related to Cicatricial Pemphigoid:

38
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053718.7ITGA6, DST, SERPINH1
2MP:00053818.5SERPINH1, DST, ITGA6, ITGB4
3MP:00053678.3CD79A, ITGB4, ITGA6, DST
4MP:00053878.3CD79A, ITGB4, ITGA6, CIITA
5MP:00053977.9CIITA, DST, ITGA6, CD79A
6MP:00036317.6SERPINH1, DST, ITGA6, ITGB4, CD79A

Publications for Cicatricial Pemphigoid

About this section
Sources:
53PubMed
See all sources

Articles related to Cicatricial Pemphigoid:

(show top 50)    (show all 419)
idTitleAuthorsYear
1
Profile of local interleukin expression in a cohort of ocular cicatricial pemphigoid patients. (23081979)
2012
2
Cicatricial pemphigoid in accompany with rheumatoid arthritis: a case report. (21180281)
2010
3
Early onset childhood cicatricial pemphigoid: a case report and review of the literature. (20537062)
2010
4
Combination treatment of intravenous immunoglobulin and cultivated oral mucosal epithelial transplantation for ocular cicatricial pemphigoid. (21686527)
2009
5
Cyclophosphamide and amniotic membrane transplantation in the management of ocular disease in a case of antiepiligrin cicatricial pemphigoid. (19747320)
2009
6
Two cases of anti-epiligrin cicatricial pemphigoid with and without associated malignancy. (18779887)
2008
7
A recalcitrant case of cicatricial pemphigoid. (17406047)
2007
8
Ocular involvement in anti-epiligrin cicatricial pemphigoid. (17191196)
2006
9
Cicatricial pemphigoid with widespread bullous eruption. (17040507)
2006
10
Ocular cicatricial pemphigoid. (16954792)
2006
11
A new method for measuring progression in patients with ocular cicatricial pemphigoid. (14769592)
2004
12
Intravenous immunoglobulin therapy in patients with ocular-cicatricial pemphigoid: a long-term follow-up. (15234140)
2004
13
Cytokine profile and supposed contribution to scarring in cicatricial pemphigoid. (12558956)
2003
14
Anti-epiligrin cicatricial pemphigoid and epidermolysis bullosa acquisita: differentiation by use of indirect immunofluorescence microscopy. (12664017)
2003
15
Chronic cicatrizing conjunctivitis in a patient with ocular cicatricial pemphigoid and fatal Wegener granulomatosis. (11730661)
2001
16
Cicatricial pemphigoid--a case report. (17664749)
2001
17
Management and outcome of cataract surgery in ocular cicatricial pemphigoid. (10766278)
2000
18
Image interpretation session: 1999. Benign mucous membrane pemphigoid (cicatricial pemphigoid). (10682788)
2000
19
Cicatricial pemphigoid diagnosed by the use of indirect immunofluorescence. (11231199)
2000
20
Intravenous immunoglobulin therapy for ocular cicatricial pemphigoid: a preliminary study. (10571350)
1999
21
Anti-epiligrin cicatricial pemphigoid with antibodies against the gamma2 subunit of laminin 5. (10052402)
1999
22
Linear IgA dermatosis mimicking ocular cicatricial pemphigoid. (10555732)
1999
23
The association between ocular cicatricial pemphigoid and rheumatoid arthritis. (9756444)
1998
24
Desquamative gingivitis and balanitis--linear IgA disease or cicatricial pemphigoid? (9894362)
1998
25
Clear corneal cataract surgery in ocular cicatricial pemphigoid. (9100121)
1997
26
Conjunctival scarring after eyelid surgery as first sign of ocular cicatricial pemphigoid. (9076789)
1997
27
Ocular cicatricial pemphigoid and ocular manifestations of pemphigus vulgaris. (9269598)
1997
28
Stenosis of the esophagus in cicatricial pemphigoid resolved by colon interposition: report of a case. (9068104)
1997
29
Management of airway obstruction in cicatricial pemphigoid. (8699892)
1996
30
Cicatricial pemphigoid: diagnosis and treatment. (8778068)
1996
31
Oesophageal webs associated with cicatricial pemphigoid. (8565261)
1995
32
Retractor plication for lower lid entropion in ocular cicatricial pemphigoid. (8333940)
1993
33
Vulvar cicatricial pemphigoid as a lichen sclerosus imitator. A case report. (1619612)
1992
34
Results of buccal mucosal grafting for patients with medically controlled ocular cicatricial pemphigoid. (1565450)
1992
35
Salt-split human skin substrate for the immunofluorescent screening of serum from patients with cicatricial pemphigoid and a new method of immunoprecipitation with IgA antibodies. (1869683)
1991
36
Obstructive respiratory failure in cicatricial pemphigoid. (2672409)
1989
37
Mast cells in conjunctiva affected by cicatricial pemphigoid. (2671849)
1989
38
The use of chemically split tissue in the detection of circulating anti-basement membrane zone antibodies in bullous pemphigoid and cicatricial pemphigoid. (3277659)
1988
39
Cicatricial pemphigoid. A case of onset at age 5. (3511219)
1986
40
Scanning electron microscopy of conjunctival surfaces in patients with ocular cicatricial pemphigoid. (3535522)
1986
41
Ocular cicatricial pemphigoid with granular IgA and complement deposition. (3904684)
1985
42
Occurrence of cicatricial pemphigoid and leiomyosarcoma in a psoriatic patient. (3891438)
1985
43
Cicatricial pemphigoid of the vulva. (3895957)
1985
44
Cicatricial pemphigoid and erythema multiforme. (6337967)
1983
45
Juvenile cicatricial pemphigoid with persistent bullous eruption: a new entity? (6754929)
1982
46
A case of cicatricial pemphigoid or cicatricial linear IgA bullous dermatosis. (6988474)
1980
47
Ocular cicatricial pemphigoid associated with practolol therapy. (773404)
1976
48
Cicatricial pemphigoid. Immunofluorescent studies. (4212995)
1974
49
Letter: Cicatricial pemphigoid. (4814935)
1974
50
CICATRICIAL PEMPHIGOID. (14214789)
1964

Variations for Cicatricial Pemphigoid

About this section

Expression for genes affiliated with Cicatricial Pemphigoid

About this section
Sources:
2BioGPS, 16Gene Expression Omnibus DataSets
See all sources
Expression patterns in normal tissues for genes affiliated with Cicatricial Pemphigoid

Search GEO for disease gene expression data for Cicatricial Pemphigoid.

Pathways for genes affiliated with Cicatricial Pemphigoid

About this section
Sources:
51PathCards, 39NCBI BioSystems Database, 56Reactome, 54QIAGEN, 5Cell Signaling Technology, 31KEGG, 61Thomson Reuters
See all sources

Pathways related to Cicatricial Pemphigoid according to GeneCards/GeneDecks:

(show all 17)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
19.5ITGA6, ITGB4
2
Show member pathways
Proteogylcan syndecan-mediated signaling events39
9.5ITGA6, ITGB4
39.5ITGB4, ITGA6
49.5ITGB4, ITGA6
5
Show member pathways
Arrhythmogenic right ventricular cardiomyopathy39
9.5ITGB4, ITGA6
69.5ITGB4, ITGA6
79.5ITGB4, ITGA6
89.5ITGA6, ITGB4
9
Show member pathways
9.5ITGA6, CIITA
109.2CD79A, CIITA
119.2CD79A, CIITA
12
Show member pathways
9.0ITGB4, ITGA6, DST
13
Show member pathways
9.0DST, ITGA6, ITGB4
14
Show member pathways
8.7CD79A, ITGB4, ITGA6
15
Show member pathways
8.7ITGA6, ITGB4, CD79A
16
Show member pathways
8.4ITGB4, ITGA6, DST, SERPINH1
17
Show member pathways
8.4ITGB4, ITGA6, DST, SERPINH1

Compounds for genes affiliated with Cicatricial Pemphigoid

About this section
Sources:
46Novoseek, 3BitterDB, 52PharmGKB, 30IUPHAR, 12DrugBank, 25HMDB, 62Tocris Bioscience
See all sources

Compounds related to Cicatricial Pemphigoid according to GeneCards/GeneDecks:

(show all 13)
idCompoundScoreTop Affiliating Genes
1enterotoxin469.4CD79A, CIITA
2allergens469.4CIITA, CD79A
3chloroquine46 3 52 30 1213.3CIITA, CD79A
4sodium dodecylsulfate469.3CD79A, CIITA
5kalinin469.3DST, ITGA6, ITGB4
6thyroxine46 2510.1CIITA, CD79A
7nitric oxide46 25 1211.0ITGB4, CIITA, SERPINH1
8mannose468.9CIITA, CD79A
9cyclosporin a46 30 6210.8CD79A, SERPINH1, CIITA
10retinoic acid46 259.7ITGA6, ITGB4, CD79A
11tyrosine468.7CIITA, SERPINH1, ITGA6, ITGB4
12oligonucleotide468.6CIITA, SERPINH1, DST, ITGA6
13vegf468.6CD79A, ITGA6, SERPINH1

GO Terms for genes affiliated with Cicatricial Pemphigoid

About this section
Sources:
17Gene Ontology
See all sources

Cellular components related to Cicatricial Pemphigoid according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1cell leading edgeGO:0312529.5ITGB4, DST
2hemidesmosomeGO:0300569.0ITGB4, ITGA6, DST
3basal plasma membraneGO:0099258.9DST, ITGA6, ITGB4
4basement membraneGO:0056048.7ITGB4, ITGA6, DST

Biological processes related to Cicatricial Pemphigoid according to GeneCards/GeneDecks:

(show all 8)
idNameGO IDScoreTop Affiliating Genes
1cell motilityGO:0488709.6DST, ITGB4
2filopodium assemblyGO:0468479.5ITGB4, ITGA6
3cell junction assemblyGO:0343299.5ITGB4, ITGA6
4response to woundingGO:0096119.4ITGB4, DST
5cell-matrix adhesionGO:0071609.2ITGB4, ITGA6
6hemidesmosome assemblyGO:0315819.2ITGB4, ITGA6, DST
7integrin-mediated signaling pathwayGO:0072299.1ITGB4, ITGA6, DST
8extracellular matrix organizationGO:0301988.5SERPINH1, DST, ITGA6, ITGB4

Molecular functions related to Cicatricial Pemphigoid according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein C-terminus bindingGO:0080229.5DST, CIITA
2integrin bindingGO:0051789.1ITGA6, DST

Products for genes affiliated with Cicatricial Pemphigoid

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Cicatricial Pemphigoid

About this section
4CDC
14ExPASy
15FMA
23GTR
24HGMD
25HMDB
26ICD10
27ICD10 via Orphanet
28ICD9CM
30IUPHAR
31KEGG
36MeSH
37MESH via Orphanet
38MGI
41NCIt
42NDF-RT
45NINDS
46Novoseek
48OMIM
49OMIM via Orphanet
53PubMed
54QIAGEN
60SNOMED-CT via Orphanet
63UMLS
64UMLS via Orphanet