MCID: PMP002
MIFTS: 36

Pemphigoid Gestationis malady

Rare diseases, Skin diseases categories

Summaries for Pemphigoid Gestationis

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NIH Rare Diseases:41 Pemphigoid gestationis (pg), also known as gestational pemphigoid or herpes gestationis, is an autoimmune skin disorder associated with pregnancy. signs and symptoms of the disorder typically present during the second or third trimester of pregnancy, but it may occur at any time during pregnancy or anywhere up to six weeks after giving birth. although the signs and symptoms are variable, a red, itchy rash with blisters is common. the rash may occur in different parts of the body, but usually develops near the belly button and/or the arms and legs. the condition is caused by the production of antibodies that attack the person's body, leading to damage and consequently blister formation. treatment generally involves systemic corticosteroids.  last updated: 8/11/2010

MalaCards based summary: Pemphigoid Gestationis, also known as gestational pemphigoid, is related to herpes gestationis and cicatricial pemphigoid, and has symptoms including premature birth, urticaria and erythema/erythematous lesions/erythroderma/polymorphous erythema. An important gene associated with Pemphigoid Gestationis is COL17A1 (collagen, type XVII, alpha 1), and among its related pathways are Allograft rejection and Degradation of the extracellular matrix. The compounds allergens and gold have been mentioned in the context of this disorder. Affiliated tissues include skin, placenta and testes.

Wikipedia:63 Gestational pemphigoid or pemphigoid gestationis (PG) is a dermatosis of pregnancy, being an autoimmune... more...

Aliases & Classifications for Pemphigoid Gestationis

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Sources:
41NIH Rare Diseases, 47Orphanet, 60UMLS, 34MESH via Orphanet, 26ICD10 via Orphanet, 61UMLS via Orphanet
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Pemphigoid Gestationis, Aliases & Descriptions:

Name: Pemphigoid Gestationis 41 47 60
Gestational Pemphigoid 41 47
 
Herpes Gestationis 41


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 47 
Rare skin diseases


External Ids:

Orphanet47 63275
MESH via Orphanet34 D006559
ICD10 via Orphanet26 L12.8
UMLS via Orphanet61 C0019343

Related Diseases for Pemphigoid Gestationis

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Diseases related to Pemphigoid Gestationis via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 43)
idRelated DiseaseScoreTop Affiliating Genes
1herpes gestationis31.0COL17A1, DST
2cicatricial pemphigoid30.9COL17A1
3pemphigus30.6DST
4bullous pemphigoid30.5DST, CCL11, COL17A1
5pemphigus vulgaris30.0CCL11, DST, HLA-DRB1
6skin disease29.9DST, CCL11, COL17A1
7choriocarcinoma10.5
8dermatitis10.5
9dermatitis herpetiformis10.5
10junctional epidermolysis bullosa10.3COL17A1
11thrombocytopenia10.2
12chorioangioma10.2
13facial hemiatrophy10.2
14cerebritis10.2
15cushing's syndrome10.2
16graves' disease10.2
17hellp syndrome10.2
18eosinophilia10.2
19pulmonary embolism10.2
20linear iga disease10.2
21malaria10.2HLA-DRB1
22systemic lupus erythematosus10.2
23lupus erythematosus10.2
24thyrotoxicosis10.2
25ectopic pregnancy10.2
26neonatal herpes10.2
27paraneoplastic syndromes10.2
28epidermolysis bullosa acquisita10.0COL17A1, DST
29epidermolysis bullosa, junctional, non-herlitz type10.0COL17A1, DST
30sarcoidosis 110.0CCL11, HLA-DRB1
31bullous skin disease10.0COL17A1, DST
32pemphigus foliaceus10.0DST, HLA-DRB1
33epidermolysis bullosa10.0COL17A1, DST
34diabetes mellitus, insulin-dependent10.0CCL11, HLA-DRB1
35epidermolysis bullosa simplex10.0COL17A1, DST
36lichen planus10.0COL17A1, DST
37ulcerative colitis10.0HLA-DRB1, CCL11
38multiple sclerosis, disease progression, modifier of10.0CCL11, HLA-DRB1
39basal cell carcinoma10.0COL17A1, DST
40galactosemia9.8CCL11, HLA-DRB1
41psoriasis9.7CCL11, DST, HLA-DRB1
42melanoma9.7HLA-DRB1, DST, CCL11
43hypersensitivity reaction type ii disease9.5COL17A1, CCL11, DST, HLA-DRB1

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



Diseases related to pemphigoid gestationis

Symptoms for Pemphigoid Gestationis

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Symptoms:

 47
  • erythema/erythematous lesions/erythroderma/polymorphous erythema
  • prematurity
  • failure to thrive/difficulties for feeding in infancy/growth delay
  • urticaria

HPO human phenotypes related to Pemphigoid Gestationis:

id Description Frequency HPO Source Accession
1 premature birth hallmark (90%) HP:0001622
2 urticaria occasional (7.5%) HP:0001025

Drugs & Therapeutics for Pemphigoid Gestationis

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Drug clinical trials:

Search ClinicalTrials for Pemphigoid Gestationis

Search NIH Clinical Center for Pemphigoid Gestationis

Genetic Tests for Pemphigoid Gestationis

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Anatomical Context for Pemphigoid Gestationis

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MalaCards organs/tissues related to Pemphigoid Gestationis:

31
Skin, Placenta, Testes, T cells

Animal Models for Pemphigoid Gestationis or affiliated genes

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Publications for Pemphigoid Gestationis

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Articles related to Pemphigoid Gestationis:

(show top 50)    (show all 102)
idTitleAuthorsYear
1
Positive clinical outcome with IVIg as monotherapy in recurrent pemphigoid gestationis. (25765353)
2015
2
A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen. (23082779)
2014
3
Recognizing presentations of pemphigoid gestationis: a case study. (25506008)
2014
4
Pemphigoid gestationis with prepartum flare. (25039537)
2014
5
Pemphigoid gestationis (herpes gestationis). (25404804)
2014
6
Successfully treated case of pemphigoid gestationis with post-partum intravenous and oral steroids. (25404796)
2014
7
Intravenous Immunoglobulin Therapy for Persistent Pemphigoid Gestationis with Steroid Induced Iatrogenic Cushing's Syndrome. (25324672)
2014
8
Pemphigoid gestationis with IgG autoantibodies to both the 120A kDa LAD-1 and the BP180 NC16a domain. (25547184)
2014
9
Pemphigoid gestationis after spontaneous expulsion of a massive complete hydatidiform mole. (24083041)
2013
10
Pemphigoid gestationis with postpartum flare-up: a case report. (24568052)
2013
11
Pemphigoid gestationis in a female with progressive facial hemiatrophy: microchimerism as a speculative shared background is disputable. (22535610)
2012
12
Successful treatment of a severe persistent case of pemphigoid gestationis with antepartum and postpartum intravenous immunoglobulin followed by azathioprine. (22309336)
2012
13
Emergency caesarean delivery of a parturient with undiagnosed pemphigoid gestationis. (22626993)
2012
14
Refractory pemphigoid gestationis postpartum. (22360419)
2012
15
Pemphigoid gestationis: pathogenesis and clinical features. (21605810)
2011
16
Pemphigoid gestationis. (21163161)
2010
17
Pemphigoid gestationis: early onset and blister formation are associated with adverse pregnancy outcomes. (19298272)
2009
18
Measurement of pruritus in a Chinese woman with pemphigoid gestationis using a wrist movement detector. (18173607)
2008
19
Enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence testing in a bullous pemphigoid and pemphigoid gestationis. (19126009)
2008
20
Severe persistent pemphigoid gestationis: long-term remission with rituximab. (17553047)
2007
21
IgG4 as the predominant IgG subclass in pemphigoides gestationis. (16630180)
2006
22
Immunoadsorption against two distinct epitopes on human type XVII collagen abolishes dermal-epidermal separation induced in vitro by autoantibodies from pemphigoid gestationis patients. (16552711)
2006
23
Pemphigoid gestationis: report of a case with umbilical artery Doppler assessment. (16913554)
2006
24
Usefulness of BP180 NC16a enzyme-linked immunosorbent assay in the serodiagnosis of pemphigoid gestationis and in differentiating between pemphigoid gestationis and pruritic urticarial papules and plaques of pregnancy. (15967916)
2005
25
Remarkable follow-up experiences of a severe persistent case of pemphigoid gestationis. (16045710)
2005
26
Pemphigoid gestationis post partum after changing husband. (16409278)
2005
27
T cell receptor gene usage of BP180-specific T lymphocytes from patients with bullous pemphigoid and pemphigoid gestationis. (15451475)
2004
28
Intravenous immune globulin in the treatment of persistent pemphigoid gestationis. (15583609)
2004
29
Tissue eosinophilia in pemphigoid gestationis: association with eotaxin and upregulated activation markers on transmigrated eosinophils. (14725564)
2004
30
The role of T lymphocytes and cytokines in the pathogenesis of pemphigoid gestationis. (12828741)
2003
31
Chronic pemphigoid gestationis: comparative clinical and immunopathological study of 10 patients. (12592077)
2003
32
Pemphigoid gestationis: treatment with immunoapheresis. (16285180)
2003
33
Pemphigoid gestationis: maternal sera recognize epitopes restricted to the N-terminal portion of the extracellular domain of BP180 not present on its shed ectodomain. (12932258)
2003
34
Successful treatment with doxycycline and nicotinamide of two cases of persistent pemphigoid gestationis. (12227878)
2002
35
Pemphigoid gestationis: new insights into the pathogenesis lead to novel diagnostic tools. (12269691)
2002
36
Pemphigoid gestationis with predominant involvement of oral mucous membranes and IgA autoantibodies targeting the C-terminus of BP180. (12399776)
2002
37
A severe persistent case of recurrent pemphigoid gestationis successfully treated with minocycline and nicotinamide. (11722465)
2001
38
Pemphigoid gestationis: a review. (10942491)
2000
39
Pemphigoid gestationis with intra-uterine death associated with foetal cerebral haemorrhage in the mid-trimester. (11012608)
2000
40
Clinical features and management of 87 patients with pemphigoid gestationis. (10457123)
1999
41
IgG1 and IgG3 are the major immunoglobulin subclasses targeting epitopes within the NC16A domain of BP180 in pemphigoid gestationis. (10417635)
1999
42
A typical case of pemphigoid gestationis with a unique pattern of intercellular immunofluorescence. (9068742)
1997
43
Pemphigoid gestationis with autoantibodies exclusively directed to the 230-kDa bullous pemphigoid antigen (BP230Ag) (8731696)
1996
44
Class II MHC typing in pemphigoid gestationis. (8565245)
1995
45
Pemphigoid gestationis and neonatal alloimmune thrombocytopenia. (2189606)
1990
46
Western blot analysis of the antigen in pemphigoid gestationis. (2186774)
1990
47
Antigen-presenting cells in the skin and placenta in pemphigoid gestationis. (2191709)
1990
48
Pemphigoid gestationis: placental interactions. (2701703)
1989
49
Immunopathology of the placenta in pemphigoid gestationis and linear IgA disease. (2667614)
1989
50
Expression of a pemphigoid gestationis-related antigen by human placenta. (3293646)
1988

Variations for Pemphigoid Gestationis

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Expression for genes affiliated with Pemphigoid Gestationis

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Search GEO for disease gene expression data for Pemphigoid Gestationis.

Pathways for genes affiliated with Pemphigoid Gestationis

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Compounds for genes affiliated with Pemphigoid Gestationis

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43Novoseek
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Compounds related to Pemphigoid Gestationis according to GeneCards Suite gene sharing:

idCompoundScoreTop Affiliating Genes
1allergens439.2CCL11, HLA-DRB1
2gold438.8DST, COL17A1
3tyrosine438.8COL17A1, CCL11, HLA-DRB1
4nacl438.5COL17A1, CCL11, DST

GO Terms for genes affiliated with Pemphigoid Gestationis

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Cellular components related to Pemphigoid Gestationis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1basement membraneGO:00056049.1COL17A1, DST
2hemidesmosomeGO:00300568.8COL17A1, DST

Biological processes related to Pemphigoid Gestationis according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1immune responseGO:00069559.3CCL11, HLA-DRB1
2hemidesmosome assemblyGO:00315819.1COL17A1, DST
3cytoskeleton organizationGO:00070108.9CCL11, DST
4extracellular matrix organizationGO:00301988.8COL17A1, DST

Products for genes affiliated with Pemphigoid Gestationis

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Sources for Pemphigoid Gestationis

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
33MeSH
34MESH via Orphanet
35MGI
38NCIt
39NDF-RT
42NINDS
43Novoseek
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46OMIM via Orphanet
50PubMed
51QIAGEN
56SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet