MCID: PMP002
MIFTS: 41

Pemphigoid Gestationis malady

Rare diseases, Skin diseases categories
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Summaries for Pemphigoid Gestationis

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NIH Rare Diseases:42 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 pemphigus, and has symptoms including erythema/erythematous lesions/erythroderma/polymorphous erythema, prematurity and failure to thrive/difficulties for feeding in infancy/growth delay. 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:65 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:
42NIH Rare Diseases, 62UMLS, 48Orphanet, 35MESH via Orphanet, 26ICD10 via Orphanet, 63UMLS via Orphanet
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Pemphigoid Gestationis, Aliases & Descriptions:

Name: Pemphigoid Gestationis 42 48 62
Gestational Pemphigoid 42 48
 
Herpes Gestationis 42 62


Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Rare diseases
Anatomical: Skin diseases
Orphanet: 48 
Rare skin diseases


External Ids:

MESH via Orphanet35 D006559
ICD10 via Orphanet26 L12.8
UMLS via Orphanet63 C0019343

Related Diseases for Pemphigoid Gestationis

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

(show all 45)
idRelated DiseaseScoreTop Affiliating Genes
1herpes gestationis31.0DST, COL17A1
2pemphigus30.6DST
3bullous pemphigoid30.5COL17A1, CCL11, DST
4pemphigus vulgaris30.0DST, HLA-DRB1, CCL11
5skin disease29.9CCL11, COL17A1, DST
6choriocarcinoma10.5
7dermatitis10.5
8dermatitis herpetiformis10.5
9mucous membrane pemphigoid10.4COL17A1
10junctional epidermolysis bullosa10.4COL17A1
11thrombocytopenia10.2
12chorioangioma10.2
13facial hemiatrophy10.2
14hellp syndrome10.2
15cicatricial pemphigoid10.2
16gestational diabetes10.2
17cushing's syndrome10.2
18graves' disease10.2
19cerebritis10.2
20eosinophilia10.2
21pulmonary embolism10.2
22linear iga disease10.2
23malaria10.2HLA-DRB1
24systemic lupus erythematosus10.2
25lupus erythematosus10.2
26thyrotoxicosis10.2
27ectopic pregnancy10.2
28neonatal herpes10.2
29paraneoplastic syndromes10.2
30epidermolysis bullosa acquisita10.0COL17A1, DST
31junctional epidermolysis bullosa, non-herlitz type10.0DST, COL17A1
32sarcoidosis10.0HLA-DRB1, CCL11
33bullous skin disease10.0DST, COL17A1
34pemphigus foliaceus10.0DST, HLA-DRB1
35epidermolysis bullosa10.0DST, COL17A1
36type 1 diabetes mellitus10.0HLA-DRB1, CCL11
37epidermolysis bullosa simplex10.0DST, COL17A1
38lichen planus10.0DST, COL17A1
39ulcerative colitis10.0CCL11, HLA-DRB1
40multiple sclerosis10.0HLA-DRB1, CCL11
41basal cell carcinoma10.0DST, COL17A1
42galactosemia9.8HLA-DRB1, CCL11
43psoriasis9.7CCL11, DST, HLA-DRB1
44melanoma9.7HLA-DRB1, DST, CCL11
45hypersensitivity reaction type ii disease9.5HLA-DRB1, DST, CCL11, COL17A1

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:

48
  • 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:

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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:

32
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 97)
idTitleAuthorsYear
1
A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen. (23082779)
2014
2
Pemphigoid gestationis with prepartum flare. (25039537)
2014
3
Pemphigoid gestationis (herpes gestationis). (25404804)
2014
4
Successfully treated case of pemphigoid gestationis with post-partum intravenous and oral steroids. (25404796)
2014
5
Intravenous Immunoglobulin Therapy for Persistent Pemphigoid Gestationis with Steroid Induced Iatrogenic Cushing's Syndrome. (25324672)
2014
6
Pemphigoid gestationis after spontaneous expulsion of a massive complete hydatidiform mole. (24083041)
2013
7
Pemphigoid gestationis with postpartum flare-up: a case report. (24568052)
2013
8
Pemphigoid gestationis in a female with progressive facial hemiatrophy: microchimerism as a speculative shared background is disputable. (22535610)
2012
9
Successful treatment of a severe persistent case of pemphigoid gestationis with antepartum and postpartum intravenous immunoglobulin followed by azathioprine. (22309336)
2012
10
Emergency caesarean delivery of a parturient with undiagnosed pemphigoid gestationis. (22626993)
2012
11
Refractory pemphigoid gestationis postpartum. (22360419)
2012
12
Pemphigoid gestationis: pathogenesis and clinical features. (21605810)
2011
13
Pemphigoid gestationis. (21163161)
2010
14
Pemphigoid gestationis: early onset and blister formation are associated with adverse pregnancy outcomes. (19298272)
2009
15
Measurement of pruritus in a Chinese woman with pemphigoid gestationis using a wrist movement detector. (18173607)
2008
16
Enzyme-linked immunosorbent assay (ELISA) and indirect immunofluorescence testing in a bullous pemphigoid and pemphigoid gestationis. (19126009)
2008
17
Severe persistent pemphigoid gestationis: long-term remission with rituximab. (17553047)
2007
18
IgG4 as the predominant IgG subclass in pemphigoides gestationis. (16630180)
2006
19
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
20
Pemphigoid gestationis: report of a case with umbilical artery Doppler assessment. (16913554)
2006
21
Clinical experience in pemphigoid gestationis: report of 10 cases. (17052488)
2006
22
Herpes gestationis (Pemphigoid gestationis). (16487884)
2006
23
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
24
Remarkable follow-up experiences of a severe persistent case of pemphigoid gestationis. (16045710)
2005
25
Pemphigoid gestationis post partum after changing husband. (16409278)
2005
26
T cell receptor gene usage of BP180-specific T lymphocytes from patients with bullous pemphigoid and pemphigoid gestationis. (15451475)
2004
27
Intravenous immune globulin in the treatment of persistent pemphigoid gestationis. (15583609)
2004
28
Tissue eosinophilia in pemphigoid gestationis: association with eotaxin and upregulated activation markers on transmigrated eosinophils. (14725564)
2004
29
The role of T lymphocytes and cytokines in the pathogenesis of pemphigoid gestationis. (12828741)
2003
30
Chronic pemphigoid gestationis: comparative clinical and immunopathological study of 10 patients. (12592077)
2003
31
Pemphigoid gestationis: treatment with immunoapheresis. (16285180)
2003
32
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
33
Successful treatment with doxycycline and nicotinamide of two cases of persistent pemphigoid gestationis. (12227878)
2002
34
Pemphigoid gestationis: new insights into the pathogenesis lead to novel diagnostic tools. (12269691)
2002
35
Pemphigoid gestationis with predominant involvement of oral mucous membranes and IgA autoantibodies targeting the C-terminus of BP180. (12399776)
2002
36
A severe persistent case of recurrent pemphigoid gestationis successfully treated with minocycline and nicotinamide. (11722465)
2001
37
Pemphigoid gestationis: a review. (10942491)
2000
38
Pemphigoid gestationis with intra-uterine death associated with foetal cerebral haemorrhage in the mid-trimester. (11012608)
2000
39
Clinical features and management of 87 patients with pemphigoid gestationis. (10457123)
1999
40
IgG1 and IgG3 are the major immunoglobulin subclasses targeting epitopes within the NC16A domain of BP180 in pemphigoid gestationis. (10417635)
1999
41
A typical case of pemphigoid gestationis with a unique pattern of intercellular immunofluorescence. (9068742)
1997
42
Pemphigoid gestationis with autoantibodies exclusively directed to the 230-kDa bullous pemphigoid antigen (BP230Ag) (8731696)
1996
43
Conversion of pemphigoid gestationis to bullous pemphigoid--two refractory cases highlighting this association. (8915153)
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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Expression patterns in normal tissues for genes affiliated with Pemphigoid Gestationis

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Pathways for genes affiliated with Pemphigoid Gestationis

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Compounds for genes affiliated with Pemphigoid Gestationis

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Sources:
44Novoseek
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Compounds related to Pemphigoid Gestationis according to GeneCards/GeneDecks:

idCompoundScoreTop Affiliating Genes
1allergens449.2CCL11, HLA-DRB1
2gold448.8COL17A1, DST
3tyrosine448.8HLA-DRB1, CCL11, COL17A1
4nacl448.5DST, COL17A1, CCL11

GO Terms for genes affiliated with Pemphigoid Gestationis

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

idNameGO IDScoreTop Affiliating Genes
1basement membraneGO:0056049.1COL17A1, DST
2hemidesmosomeGO:0300568.8COL17A1, DST

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

idNameGO IDScoreTop Affiliating Genes
1immune responseGO:0069559.3CCL11, HLA-DRB1
2hemidesmosome assemblyGO:0315819.1COL17A1, DST
3cytoskeleton organizationGO:0070108.9CCL11, DST
4extracellular matrix organizationGO:0301988.8COL17A1, 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
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet