MCID: PMP002
MIFTS: 40

Pemphigoid Gestationis

Categories: Immune diseases, Rare diseases, Skin diseases
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Aliases & Classifications for Pemphigoid Gestationis

MalaCards integrated aliases for Pemphigoid Gestationis:

Name: Pemphigoid Gestationis 11 19 58 43 14 16 71 33
Herpes Gestationis 11 19 53 31 33
Gestational Pemphigoid 19 58 75 33
Gestational Herpes 11

Characteristics:


Prevelance:

1-9/1000000 (Germany, Kuwait, Germany) <1/1000000 (France) 58

Age Of Onset:

Adolescent,Adult 58

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

Disease Ontology 11 DOID:14482
MeSH 43 D006559
NCIt 49 C85003
SNOMED-CT 68 86081009
ICD10 31 O26.4
MESH via Orphanet 44 D006559
ICD10 via Orphanet 32 L12.8
UMLS via Orphanet 72 C0019343
Orphanet 58 ORPHA63275
ICD11 33 1405755890
UMLS 71 C0019343

Summaries for Pemphigoid Gestationis

GARD: 19 Pemphigoid gestationis (PG) is a pregnancy-associated, autoimmune skin disorder. It usually begins abruptly during the 2nd or 3rd trimester of pregnancy, but it can begin at any time during pregnancy. Symptoms often include the sudden formation of very itchy, red bumps and/or blisters on the abdomen and trunk, which may then spread to other parts of the body. Unrelenting itchiness (pruritus) often interferes with daily activities. Symptoms may improve at the end of pregnancy, but flares may occur during, or right after, delivery. PG is caused by a woman's immune system producing autoantibodies and mistakenly attacking her own skin, but the trigger for autoantibody production is poorly understood.

MalaCards based summary: Pemphigoid Gestationis, also known as herpes gestationis, is related to pemphigus gestationis and autoimmune disease, and has symptoms including pruritis and morning sickness. An important gene associated with Pemphigoid Gestationis is COL17A1 (Collagen Type XVII Alpha 1 Chain), and among its related pathways/superpathways are COPI-independent Golgi-to-ER retrograde traffic and Keratinization. Affiliated tissues include skin, placenta and t cells, and related phenotypes are premature birth and pruritus

Disease Ontology: 11 A pemphigoid that is characterized by erythematous papules, vesicles, and plaques around the trunk and extremities that develop during pregnancy, particularly during the second and third trimesters, has symptom pruritis, and has material basis in autoantibodies to the basement membrane, which triggers a complement cascade and attracts eosinophils to the subepidermis.

Orphanet: 58 A rare autoimmune bullous skin disease characterized by pruritus with or without polymorphic skin eruption, affecting pregnant women typically during the second and third trimester.

Wikipedia: 75 Gestational pemphigoid (GP) is a rare autoimmune variant of the skin disease bullous pemphigoid, and... more...

Related Diseases for Pemphigoid Gestationis

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

(show top 50) (show all 141)
# Related Disease Score Top Affiliating Genes
1 pemphigus gestationis 30.7 DST DSG3 COL17A1
2 autoimmune disease 30.5 HLA-DRB1 DSG3 DSG1 COL17A1
3 pompholyx 30.4 DST COL17A1
4 epidermolysis bullosa acquisita 30.2 HLA-DRB1 DST COL17A1
5 cicatricial pemphigoid 30.1 DST DSG3 DSG1 COL17A1
6 pemphigoid 30.1 RNASE3 PPL EVPL DST DSG3 DSG1
7 lichen planus 29.9 DST DSG3 DSG1 COL17A1
8 skin disease 29.9 RNASE3 HLA-DRB1 DST DSG3 DSG1 COL17A1
9 celiac disease 1 29.8 TNFRSF25 TNFRSF10A HLA-DRB1
10 bullous pemphigoid 29.8 RNASE3 PPL HLA-DRB1 EVPL DST DSG3
11 subcorneal pustular dermatosis 29.1 PPL EVPL DST DSG3 DSG1
12 pemphigus 28.8 PPL HLA-DRB1 EVPL DST DSG3 DSG1
13 pemphigus foliaceus 28.7 PPL HLA-DRB1 EVPL DST DSG3 DSG1
14 pemphigus vulgaris, familial 28.6 PPL HLA-DRB1 EVPL DST DSG3 DSG1
15 paraneoplastic pemphigus 28.6 PPL EVPL DST DSG3 DSG1 COL17A1
16 bullous skin disease 28.6 PPL EVPL DST DSG3 DSG1 COL17A1
17 pruritic urticarial papules and plaques of pregnancy 10.5
18 dermatitis herpetiformis 10.5
19 exanthem 10.4
20 gestational trophoblastic neoplasm 10.3
21 neurotic excoriation 10.2 DST COL17A1
22 epidermolysis bullosa, junctional 4, intermediate 10.2 DST COL17A1
23 scabies 10.2 DST COL17A1
24 mite infestation 10.2 DST COL17A1
25 junctional epidermolysis bullosa non-herlitz type 10.2 DST COL17A1
26 epidermolysis bullosa, junctional 5b, with pyloric atresia 10.2 DST COL17A1
27 recessive dystrophic epidermolysis bullosa 10.2 DST COL17A1
28 leukocyte adhesion deficiency, type i 10.2
29 hydatidiform mole, recurrent, 1 10.2
30 graves disease 1 10.2
31 erythema multiforme 10.2
32 intrahepatic cholestasis of pregnancy 10.2
33 pre-eclampsia 10.2
34 gestational diabetes 10.2
35 graves' disease 10.2
36 cholestasis 10.2
37 urticaria 10.2
38 intrahepatic cholestasis 10.2
39 dermatitis 10.2
40 choriocarcinoma 10.2
41 lichen planus pemphigoides 10.2
42 kindler syndrome 10.2 DST COL17A1
43 epidermolysis bullosa dystrophica 10.1 DST COL17A1
44 palindromic rheumatism 10.1 TNFRSF10A HLA-DRB1
45 hirata disease 10.1 TNFRSF10A HLA-DRB1
46 acrodermatitis 10.1
47 alopecia 10.1
48 alopecia totalis 10.1
49 congenital herpes simplex 10.1
50 focal epithelial hyperplasia 10.1 TNFRSF10A HLA-DRB1

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



Diseases related to Pemphigoid Gestationis

Symptoms & Phenotypes for Pemphigoid Gestationis

Human phenotypes related to Pemphigoid Gestationis:

58 30 (show all 6)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 premature birth 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0001622
2 pruritus 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0000989
3 skin vesicle 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0200037
4 abnormal blistering of the skin 58 30 Hallmark (90%) Very frequent (99-80%)
HP:0008066
5 failure to thrive 58 30 Frequent (33%) Frequent (79-30%)
HP:0001508
6 intrauterine growth retardation 58 30 Frequent (33%) Frequent (79-30%)
HP:0001511

Symptoms:

11
  • pruritis

UMLS symptoms related to Pemphigoid Gestationis:


morning sickness

MGI Mouse Phenotypes related to Pemphigoid Gestationis:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.1 COL17A1 DSG1 DSG3 DST EVPL PPL

Drugs & Therapeutics for Pemphigoid Gestationis

Search Clinical Trials, NIH Clinical Center for Pemphigoid Gestationis

Cochrane evidence based reviews: pemphigoid gestationis

Genetic Tests for Pemphigoid Gestationis

Anatomical Context for Pemphigoid Gestationis

Organs/tissues related to Pemphigoid Gestationis:

MalaCards : Skin, Placenta, T Cells, Ovary, Thyroid

Publications for Pemphigoid Gestationis

Articles related to Pemphigoid Gestationis:

(show top 50) (show all 651)
# Title Authors PMID Year
1
Pemphigoid gestationis: clinical and laboratory evaluation. 53 62
19936176 2009
2
Herpes gestationis in a mother and newborn: immunoclinical perspectives based on a weekly follow-up of the enzyme-linked immunosorbent assay index of a bullous pemphigoid antigen noncollagenous domain. 53 62
17875879 2007
3
Molecular mapping of the major epitopes of BP180 recognized by herpes gestationis autoantibodies. 53 62
10479533 1999
4
Acquired skin disease of hemidesmosomes. 53 62
10379705 1999
5
Immunoreactivity of bullous pemphigoid (BP) autoantibodies against the NC16A and C-terminal domains of the 180 kDa BP antigen (BP180): immunoblot analysis and enzyme-linked immunosorbent assay using BP180 recombinant proteins. 53 62
9767278 1998
6
The majority of bullous pemphigoid and herpes gestationis serum samples react with the NC16a domain of the 180-kDa bullous pemphigoid antigen. 53 62
8874743 1996
7
Cicatricial pemphigoid autoantibodies react with multiple sites on the BP180 extracellular domain. 53 62
8592065 1996
8
Molecular mapping of a pathogenically relevant BP180 epitope associated with experimentally induced murine bullous pemphigoid. 53 62
7594563 1995
9
Serum eosinophil cationic protein (ECP) in bullous pemphigoid. 53 62
7751092 1995
10
Development of an ELISA to detect anti-BP180 autoantibodies in bullous pemphigoid and herpes gestationis. 53 62
7516396 1994
11
[Herpes gestationis: immunologic and immunogenetic aspects]. 53 62
8113038 1993
12
A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. 53 62
7693763 1993
13
Bullous pemphigoid and herpes gestationis autoantibodies recognize a common non-collagenous site on the BP180 ectodomain. 53 62
8228259 1993
14
Cloning of partial cDNA for mouse 180-kDa bullous pemphigoid antigen (BPAG2), a highly conserved collagenous protein of the cutaneous basement membrane zone. 53 62
1512460 1992
15
Isolation of a human epidermal cDNA corresponding to the 180-kD autoantigen recognized by bullous pemphigoid and herpes gestationis sera. Immunolocalization of this protein to the hemidesmosome. 53 62
1698819 1990
16
Histopathological features of pemphigoid gestationis and polymorphic eruption of pregnancy: A blinded retrospective comparative study of 31 cases. 62
36357351 2022
17
Recognizing, Diagnosing, and Managing Pregnancy Dermatoses. 62
36075066 2022
18
The association of six autoimmune bullous diseases with thyroid disorders: a population-based study. 62
35611551 2022
19
Pemphigoid gestationis and preeclampsia in a donor-egg IVF pregnancy. 62
35919284 2022
20
Acral blisters in neonates: Three rare and benign diseases. 62
36245441 2022
21
Successful treatment of recalcitrant pemphigoid gestationis with omalizumab: report of two cases. 62
35592910 2022
22
Pseudo-Pemphigoid Gestationis Eruption Following SARS-CoV-2 Vaccination with mRNA Vaccine. 62
35892478 2022
23
Pemphigoid Gestationis and adverse pregnancy outcomes: A literature review. 62
35385801 2022
24
Newborn twins with neonatal pemphigoid gestationis. 62
34939222 2022
25
Gestational Pemphigoid Presenting in the Second Trimester of Pregnancy: A Rare Finding. 62
35800842 2022
26
Pruritus in Pregnancy. 62
35191007 2022
27
[Skin and pregnancy]. 62
35353455 2022
28
Autoreactivity to BP180 Neoepitopes in Patients With Pemphigoid Gestationis. 62
34985500 2022
29
Pemphigoid gestationis and polymorphic eruption of pregnancy in skin of color. 62
35064677 2022
30
Annular bullous diseases. 62
34979265 2022
31
Type XVII collagen: Relevance of distinct epitopes, complement-independent effects, and association with neurological disorders in pemphigoid disorders. 62
36032160 2022
32
Influence of pemphigoid gestationis on pregnancy outcome: A case report and review of the literature. 62
34815775 2022
33
Diagnosis, fetal risk and treatment of pemphigoid gestationis in pregnancy: A case report. 62
35004996 2021
34
Treatment of pemphigoid gestationis with dupilumab. 62
34048080 2021
35
Assessment and management of itchy skin in pregnancy. 62
34845477 2021
36
A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey. 62
34642934 2021
37
Neonatal pemphigoid gestationis: An atypical presentation of a rare disease. 62
34713489 2021
38
The burden of neurological comorbidities in six autoimmune bullous diseases: a population-based study. 62
34153122 2021
39
Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach. 62
34304937 2021
40
Subepithelial autoimmune blistering dermatoses: Clinical features and diagnosis. 62
33684496 2021
41
Severe pemphigoid gestationis responsive to intravenous immunoglobulins after initial treatment failure of combined oral steroids and cyclosporin. 62
34001471 2021
42
Pemphigoid gestationis: a rare pregnancy dermatosis treated with a combination of IVIg and rituximab. 62
33727302 2021
43
Pemphigoid Gestationis - Case Report and Review of Literature. 62
34168478 2021
44
Intravenous Immunoglobulin Therapy for Gestational Pemphigoid Refractory to Conventional Treatment. 62
32986975 2021
45
Flame Figures in Linear Immunoglobulin A Bullous Dermatosis Secondary to Parvovirus B19 Infection. 62
32472771 2021
46
The epidemiology of autoimmune bullous diseases in Sudan between 2000 and 2016. 62
34255799 2021
47
Pemphigoid Gestationis-A Rare Pregnancy Dermatoses. 62
33417630 2020
48
A Rare Case of Recurrent Postpartum Pemphigoid Gestationis with Fetal Complications and Skip Pregnancy. 62
33344355 2020
49
Pemphigoid Gestationis. 62
32846065 2020
50
A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita. 62
32180161 2020

Variations for Pemphigoid Gestationis

Expression for Pemphigoid Gestationis

Search GEO for disease gene expression data for Pemphigoid Gestationis.

Pathways for Pemphigoid Gestationis

Pathways related to Pemphigoid Gestationis according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
11.62 PPL EVPL DST
2
Show member pathways
11.46 PPL EVPL DSG3 DSG1
3 10.89 EVPL DST

GO Terms for Pemphigoid Gestationis

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

# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 10.36 TNFRSF25 TNFRSF10A RNF103 HLA-DRB1 DST DSG3
2 membrane GO:0016021 10.36 TNFRSF25 TNFRSF10A RNF103 HLA-DRB1 DST DSG3
3 intermediate filament GO:0005882 9.92 DST EVPL HLA-DRB1 PPL
4 cornified envelope GO:0001533 9.76 PPL EVPL DSG3 DSG1
5 hemidesmosome GO:0030056 9.67 DST COL17A1
6 cellular anatomical entity GO:0110165 9.65 TNFRSF25 TNFRSF10A PPL EVPL DST
7 anchoring junction GO:0070161 9.43 PPL EVPL DST DSG3 DSG1 COL17A1
8 desmosome GO:0030057 9.23 PPL EVPL DSG3 DSG1

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

# Name GO ID Score Top Affiliating Genes
1 epidermis development GO:0008544 9.73 HLA-DRB1 EVPL COL17A1
2 wound healing GO:0042060 9.63 PPL EVPL DST
3 hemidesmosome assembly GO:0031581 9.26 DST COL17A1
4 intermediate filament cytoskeleton organization GO:0045104 9.1 PPL EVPL DST

Molecular functions related to Pemphigoid Gestationis according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural molecule activity GO:0005198 9.1 PPL EVPL DST

Sources for Pemphigoid Gestationis

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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