MCID: PMP002
MIFTS: 40

Pemphigoid Gestationis

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Pemphigoid Gestationis

MalaCards integrated aliases for Pemphigoid Gestationis:

Name: Pemphigoid Gestationis 49 55 69
Gestational Pemphigoid 49 55
Herpes Gestationis 49

Classifications:

Orphanet: 55  
Rare skin diseases


External Ids:

Orphanet 55 ORPHA63275
MESH via Orphanet 42 D006559
UMLS via Orphanet 70 C0019343
ICD10 via Orphanet 33 L12.8

Summaries for Pemphigoid Gestationis

NIH Rare Diseases : 49 Pemphigoid gestationis (PG) is a pregnancy-associated autoimmune skin disorder. It usually begins abruptly during the 2nd or 3rd trimester of pregnancy, but can occur any time during pregnancy. Signs and symptoms often include the formation of very itchy, red bumps and/or blisters on the abdomen and trunk, but other parts of the body can be affected. 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. While it usually goes away on its own within weeks to months after delivery, it may recur during menses; with use of oral contraceptives; and/or with subsequent pregnancies. PG is caused by a woman's immune system mistakenly attacking her own skin, but the exact reasons why this occurs is poorly understood. Treatment depends on severity and may include topical corticosteroids; oral corticosteroids, and/or oral antihistamines to relieve itching. Last updated: 5/3/2016

MalaCards based summary : Pemphigoid Gestationis, also known as gestational pemphigoid, is related to herpes gestationis and pemphigus, and has symptoms including pruritus, failure to thrive and intrauterine growth retardation. An important gene associated with Pemphigoid Gestationis is COL17A1 (Collagen Type XVII Alpha 1 Chain), and among its related pathways/superpathways are Allograft rejection and Hematopoietic cell lineage. Affiliated tissues include skin, placenta and t cells.

Wikipedia : 72 Gestational pemphigoid or pemphigoid gestationis (PG) is a dermatosis of pregnancy, being an autoimmune... 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 95)
# Related Disease Score Top Affiliating Genes
1 herpes gestationis 32.8 COL17A1 DST
2 pemphigus 30.2 COL17A1 DST
3 lichen planus pemphigoides 30.2 COL17A1 DST
4 linear iga disease 30.2 COL17A1 DST
5 cicatricial pemphigoid 30.1 COL17A1 DST
6 pemphigus vulgaris 30.1 DST HLA-DRB1
7 lichen planus 30.0 COL17A1 DST
8 autoimmune disease 29.3 COL17A1 HLA-DRB1 IL5
9 bullous pemphigoid 28.5 CCL11 COL17A1 DST HLA-DRB1 IL5
10 neurotic excoriation 10.2 COL17A1 DST
11 autoimmune disease of skin and connective tissue 10.2 COL17A1 DST
12 vesiculobullous skin disease 10.2 COL17A1 DST
13 epidermolysis bullosa simplex with muscular dystrophy 10.2 COL17A1 DST
14 epidermolysis bullosa junctionalis with pyloric atresia 10.2 COL17A1 DST
15 epidermolysis bullosa acquisita 10.2 COL17A1 DST
16 lichen disease 10.2 COL17A1 DST
17 ecthyma 10.2 COL17A1 DST
18 pruritic urticarial papules and plaques of pregnancy 10.2
19 subcorneal pustular dermatosis 10.2 COL17A1 DST
20 junctional epidermolysis bullosa 10.2 COL17A1 DST
21 epidermolysis bullosa, junctional, non-herlitz type 10.2 COL17A1 DST
22 lichen planopilaris 10.1 COL17A1 HLA-DRB1
23 epidermolysis bullosa simplex 10.1 COL17A1 DST
24 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.1
25 pemphigus foliaceus 10.1 DST HLA-DRB1
26 blood group, i system 10.0
27 pemphigus vulgaris, familial 10.0
28 hydatidiform mole, recurrent, 1 10.0
29 autoimmune disease 1 10.0
30 epidermolysis bullosa 10.0 COL17A1 DST
31 chronic meningitis 9.9 CCL11 IL5
32 angiostrongyliasis 9.9 CCL11 IL5
33 strongyloidiasis 9.9 CCL11 IL5
34 eosinophilic gastroenteritis 9.9 CCL11 IL5
35 chronic eosinophilic pneumonia 9.9 CCL11 IL5
36 gastrointestinal allergy 9.9 CCL11 IL5
37 eosinophilic meningitis 9.9 CCL11 IL5
38 chronic conjunctivitis 9.9 CCL11 IL5
39 eosinophilic gastritis 9.9 CCL11 IL5
40 kimura disease 9.9 CCL11 IL5
41 folliculitis 9.9 CCL11 IL5
42 vernal keratoconjunctivitis 9.9 CCL11 IL5
43 radiculopathy 9.9 CCL11 IL5
44 conjunctival disease 9.9 CCL11 IL5
45 pulmonary eosinophilia 9.9 CCL11 IL5
46 eosinophilic pneumonia 9.9 CCL11 IL5
47 onchocerciasis 9.9 CCL11 IL5
48 allergic conjunctivitis 9.9 CCL11 IL5
49 nasal cavity disease 9.9 CCL11 IL5
50 esophagitis, eosinophilic, 1 9.9 CCL11 IL5

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:

55 31 (show all 6)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 pruritus 55 31 hallmark (90%) Very frequent (99-80%) HP:0000989
2 failure to thrive 55 31 frequent (33%) Frequent (79-30%) HP:0001508
3 intrauterine growth retardation 55 31 frequent (33%) Frequent (79-30%) HP:0001511
4 premature birth 55 31 hallmark (90%) Very frequent (99-80%) HP:0001622
5 abnormal blistering of the skin 55 31 hallmark (90%) Very frequent (99-80%) HP:0008066
6 skin vesicle 55 31 hallmark (90%) Very frequent (99-80%) HP:0200037

UMLS symptoms related to Pemphigoid Gestationis:


morning sickness

Drugs & Therapeutics for Pemphigoid Gestationis

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Recruiting NCT02874079

Search NIH Clinical Center for Pemphigoid Gestationis

Genetic Tests for Pemphigoid Gestationis

Anatomical Context for Pemphigoid Gestationis

MalaCards organs/tissues related to Pemphigoid Gestationis:

38
Skin, Placenta, T Cells, Testes

Publications for Pemphigoid Gestationis

Articles related to Pemphigoid Gestationis:

(show top 50) (show all 125)
# Title Authors Year
1
Anhydramnios in Patients With Pemphigoid Gestationis. ( 29365022 )
2018
2
Early flare-up of severe Herpes gestationis (Pemphigoid gestationis) and successfull prolonged treatment: A case report. ( 28399702 )
2017
3
Pemphigoid gestationis: current perspectives. ( 29184427 )
2017
4
Sensitivity and specificity of BP180 NC16A enzyme-linked immunosorbent assay for the diagnosis of pemphigoid gestationis. ( 28212762 )
2017
5
Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder. ( 28524368 )
2017
6
Pemphigoid gestationis: Clinical and histologic features of twenty-three patients. ( 28560301 )
2017
7
Pemphigoid Gestationis ( 29261895 )
2017
8
Image Gallery: Pemphigoid gestationis mimicking a gyrate erythema. ( 28418130 )
2017
9
New Technology Identifies Pemphigoid Gestationis by Detection of Anti-BP180 IgG, but Simultaneous Detection of Anti-BP230 IgG does not Improve Diagnosis. ( 27174635 )
2016
10
Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis. ( 27416093 )
2016
11
Successful treatment of a severe early onset case of pemphigoid gestationis with intravenous immunoglobulin in a twin pregnancy conceived with in vitro fertilisation in a primigravida. ( 27922278 )
2016
12
Concomitant pemphigus vulgaris and pemphigoid gestationis: a case report and review of the literature. ( 26990471 )
2016
13
Retrospective analysis of pemphigoid gestationis in 32 Saudi patients - Clinicopathological features and a literature review. ( 27175516 )
2016
14
Pemphigoid gestationis: a successful preventive treatment by rituximab. ( 27606493 )
2016
15
Pregnancy with Pemphigoid Gestationis: A Rare Entity. ( 27630915 )
2016
16
Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis. ( 27265076 )
2016
17
Pemphigoid Gestationis: A Case Report and Review of Management. ( 26267738 )
2015
18
A severe and prolonged case of pemphigoid gestationis successfully treated with combination therapies. ( 26521896 )
2015
19
Pemphigoid Gestationis Complicating an Egg Donation Pregnancy. ( 26608214 )
2015
20
Pemphigoid gestationis. ( 25581583 )
2015
21
Dermatological Diseases Associated with Pregnancy: Pemphigoid Gestationis, Polymorphic Eruption of Pregnancy, Intrahepatic Cholestasis of Pregnancy, and Atopic Eruption of Pregnancy. ( 26609305 )
2015
22
What is your diagnosis? pemphigoid gestationis (herpes gestationis). ( 26057509 )
2015
23
Positive clinical outcome with IVIg as monotherapy in recurrent pemphigoid gestationis. ( 25765353 )
2015
24
Pemphigoid gestationis revealing a denial of pregnancy. ( 26299950 )
2015
25
Clinical and immunological profiles of 25 patients with pemphigoid gestationis. ( 25154546 )
2014
26
Pemphigoid gestationis with IgG autoantibodies to both the 120A kDa LAD-1 and the BP180 NC16a domain. ( 25547184 )
2014
27
A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen. ( 23082779 )
2014
28
Intravenous Immunoglobulin Therapy for Persistent Pemphigoid Gestationis with Steroid Induced Iatrogenic Cushing's Syndrome. ( 25324672 )
2014
29
Pemphigoid gestationis with prepartum flare. ( 25039537 )
2014
30
Pemphigoid gestationis in a third trimester pregnancy. ( 25431712 )
2014
31
Recognizing presentations of pemphigoid gestationis: a case study. ( 25506008 )
2014
32
Case for diagnosis. Pemphigoid gestationis. ( 24626668 )
2014
33
Pemphigoid gestationis (herpes gestationis). ( 25404804 )
2014
34
Successfully treated case of pemphigoid gestationis with post-partum intravenous and oral steroids. ( 25404796 )
2014
35
Pemphigoid gestationis: a retrospective study in southwest Iran. ( 23852847 )
2013
36
Pemphigoid gestationis with postpartum flare-up: a case report. ( 24568052 )
2013
37
Pemphigoid gestationis. ( 24365006 )
2013
38
Pemphigoid gestationis after spontaneous expulsion of a massive complete hydatidiform mole. ( 24083041 )
2013
39
Pemphigoid gestationis. ( 24007701 )
2013
40
Dyshidrosiform presentation (pompholyx-like) of pemphigoid gestationis with intrauterine fetal death. ( 23432199 )
2013
41
Suspicion of pulmonary embolism during treatment of pemphigoid gestationis. ( 24278048 )
2013
42
The utility of C4d immunohistochemistry on formalin-fixed paraffin-embedded tissue in the distinction of polymorphic eruption of pregnancy from pemphigoid gestationis. ( 24061402 )
2013
43
Emergency caesarean delivery of a parturient with undiagnosed pemphigoid gestationis. ( 22626993 )
2012
44
Pemphigoid gestationis. ( 22137226 )
2012
45
Pemphigoid gestationis with a complete hydatidiform mole. ( 21950371 )
2012
46
Successful treatment of a severe persistent case of pemphigoid gestationis with antepartum and postpartum intravenous immunoglobulin followed by azathioprine. ( 22309336 )
2012
47
Glucocorticosteroid-resistant pemphigoid gestationis: successful treatment with adjuvant immunoadsorption. ( 22379622 )
2012
48
Refractory pemphigoid gestationis postpartum. ( 22360419 )
2012
49
Pemphigoid gestationis in a female with progressive facial hemiatrophy: microchimerism as a speculative shared background is disputable. ( 22535610 )
2012
50
Postpartum pemphigoid gestationis. ( 22615518 )
2012

Variations for Pemphigoid Gestationis

Expression for Pemphigoid Gestationis

Search GEO for disease gene expression data for Pemphigoid Gestationis.

Pathways for Pemphigoid Gestationis

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 basement membrane GO:0005604 8.96 COL17A1 DST
2 hemidesmosome GO:0030056 8.62 COL17A1 DST

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

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 8.8 CCL11 HLA-DRB1 IL5

Sources for Pemphigoid Gestationis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....