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 53 59 73
Gestational Pemphigoid 53 59
Herpes Gestationis 53

Classifications:

Orphanet: 59  
Rare skin diseases


External Ids:

Orphanet 59 ORPHA63275
ICD10 via Orphanet 34 L12.8
UMLS via Orphanet 74 C0019343
MESH via Orphanet 45 D006559

Summaries for Pemphigoid Gestationis

NIH Rare Diseases : 53 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.

MalaCards based summary : Pemphigoid Gestationis, also known as gestational pemphigoid, is related to herpes gestationis and lichen planus pemphigoides, and has symptoms including morning sickness 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, and related phenotypes are pruritus and failure to thrive

Wikipedia : 76 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 33.7 COL17A1 DST
2 lichen planus pemphigoides 30.4 COL17A1 DST
3 linear iga disease 30.4 COL17A1 DST
4 cicatricial pemphigoid 30.3 COL17A1 DST
5 pemphigus 30.2 COL17A1 DST
6 pemphigus vulgaris 30.0 DST HLA-DRB1
7 lichen planus 30.0 COL17A1 DST
8 autoimmune disease 28.9 COL17A1 HLA-DRB1 IL5
9 bullous pemphigoid 27.4 CCL11 COL17A1 DST HLA-DRB1 IL5
10 recessive dystrophic epidermolysis bullosa-generalized other 11.2
11 neurotic excoriation 10.4 COL17A1 DST
12 vesiculobullous skin disease 10.3 COL17A1 DST
13 epidermolysis bullosa simplex with muscular dystrophy 10.3 COL17A1 DST
14 autoimmune disease of skin and connective tissue 10.3 COL17A1 DST
15 epidermolysis bullosa acquisita 10.3 COL17A1 DST
16 lichen disease 10.3 COL17A1 DST
17 epidermolysis bullosa junctionalis with pyloric atresia 10.3 COL17A1 DST
18 ecthyma 10.3 COL17A1 DST
19 subcorneal pustular dermatosis 10.3 COL17A1 DST
20 epidermolysis bullosa, junctional, non-herlitz type 10.2 COL17A1 DST
21 junctional epidermolysis bullosa 10.2 COL17A1 DST
22 lichen planopilaris 10.2 COL17A1 HLA-DRB1
23 epidermolysis bullosa simplex 10.2 COL17A1 DST
24 pruritic urticarial papules and plaques of pregnancy 10.2
25 pemphigus foliaceus 10.1 DST HLA-DRB1
26 pemphigus vulgaris, familial 10.0
27 hydatidiform mole, recurrent, 1 10.0
28 gestational trophoblastic neoplasm 10.0
29 autoimmune disease 1 10.0
30 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.0
31 epidermolysis bullosa 10.0 COL17A1 DST
32 chronic meningitis 9.9 CCL11 IL5
33 angiostrongyliasis 9.9 CCL11 IL5
34 strongyloidiasis 9.9 CCL11 IL5
35 eosinophilic gastroenteritis 9.9 CCL11 IL5
36 chronic eosinophilic pneumonia 9.9 CCL11 IL5
37 eosinophilic meningitis 9.9 CCL11 IL5
38 gastrointestinal allergy 9.9 CCL11 IL5
39 chronic conjunctivitis 9.9 CCL11 IL5
40 eosinophilic gastritis 9.9 CCL11 IL5
41 kimura disease 9.9 CCL11 IL5
42 folliculitis 9.9 CCL11 IL5
43 pompholyx 9.9
44 gestational diabetes 9.9
45 pulmonary embolism 9.9
46 conn's syndrome 9.9
47 graves' disease 9.9
48 hellp syndrome 9.9
49 cholestasis 9.9
50 facial hemiatrophy 9.9

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:

59 32 (show all 6)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 pruritus 59 32 hallmark (90%) Very frequent (99-80%) HP:0000989
2 failure to thrive 59 32 frequent (33%) Frequent (79-30%) HP:0001508
3 intrauterine growth retardation 59 32 frequent (33%) Frequent (79-30%) HP:0001511
4 premature birth 59 32 hallmark (90%) Very frequent (99-80%) HP:0001622
5 abnormal blistering of the skin 59 32 hallmark (90%) Very frequent (99-80%) HP:0008066
6 skin vesicle 59 32 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 Register of Autoimmune Bullous Dermatoses Completed NCT02899923
2 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Recruiting NCT02874079 Not Applicable

Search NIH Clinical Center for Pemphigoid Gestationis

Genetic Tests for Pemphigoid Gestationis

Anatomical Context for Pemphigoid Gestationis

MalaCards organs/tissues related to Pemphigoid Gestationis:

41
Skin, Placenta, T Cells, Testes

Publications for Pemphigoid Gestationis

Articles related to Pemphigoid Gestationis:

(show top 50) (show all 130)
# Title Authors Year
1
Pemphigoid Gestationis: A Case Presentation. ( 29486593 )
2018
2
Pemphigoid gestationis successfully treated with intravenous immunoglobulin. ( 29627782 )
2018
3
Pemphigoid gestationis with lethal fetal malformation and postpartum persistence. ( 29873913 )
2018
4
Anhydramnios in Patients With Pemphigoid Gestationis. ( 29365022 )
2018
5
Pemphigoid gestationis mit letaler fAPtaler Fehlbildung und postpartaler Persistenz. ( 29873912 )
2018
6
Pemphigoid gestationis: a case series and review of the literature. ( 29620424 )
2018
7
Pemphigoid gestationis: Clinical and histologic features of twenty-three patients. ( 28560301 )
2017
8
Early flare-up of severe Herpes gestationis (Pemphigoid gestationis) and successfull prolonged treatment: A case report. ( 28399702 )
2017
9
Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder. ( 28524368 )
2017
10
Pemphigoid gestationis: current perspectives. ( 29184427 )
2017
11
Pemphigoid Gestationis ( 29261895 )
2017
12
Sensitivity and specificity of BP180 NC16A enzyme-linked immunosorbent assay for the diagnosis of pemphigoid gestationis. ( 28212762 )
2017
13
Image Gallery: Pemphigoid gestationis mimicking a gyrate erythema. ( 28418130 )
2017
14
Concomitant pemphigus vulgaris and pemphigoid gestationis: a case report and review of the literature. ( 26990471 )
2016
15
Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis. ( 27265076 )
2016
16
Pemphigoid gestationis: a successful preventive treatment by rituximab. ( 27606493 )
2016
17
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
18
New Technology Identifies Pemphigoid Gestationis by Detection of Anti-BP180 IgG, but Simultaneous Detection of Anti-BP230 IgG does not Improve Diagnosis. ( 27174635 )
2016
19
Pregnancy with Pemphigoid Gestationis: A Rare Entity. ( 27630915 )
2016
20
Retrospective analysis of pemphigoid gestationis in 32 Saudi patients - Clinicopathological features and a literature review. ( 27175516 )
2016
21
Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis. ( 27416093 )
2016
22
Dermatological Diseases Associated with Pregnancy: Pemphigoid Gestationis, Polymorphic Eruption of Pregnancy, Intrahepatic Cholestasis of Pregnancy, and Atopic Eruption of Pregnancy. ( 26609305 )
2015
23
Pemphigoid gestationis. ( 25581583 )
2015
24
What is your diagnosis? pemphigoid gestationis (herpes gestationis). ( 26057509 )
2015
25
Positive clinical outcome with IVIg as monotherapy in recurrent pemphigoid gestationis. ( 25765353 )
2015
26
Pemphigoid gestationis revealing a denial of pregnancy. ( 26299950 )
2015
27
A severe and prolonged case of pemphigoid gestationis successfully treated with combination therapies. ( 26521896 )
2015
28
Pemphigoid Gestationis Complicating an Egg Donation Pregnancy. ( 26608214 )
2015
29
Pemphigoid Gestationis: A Case Report and Review of Management. ( 26267738 )
2015
30
Pemphigoid gestationis with prepartum flare. ( 25039537 )
2014
31
Pemphigoid gestationis in a third trimester pregnancy. ( 25431712 )
2014
32
Successfully treated case of pemphigoid gestationis with post-partum intravenous and oral steroids. ( 25404796 )
2014
33
Case for diagnosis. Pemphigoid gestationis. ( 24626668 )
2014
34
Recognizing presentations of pemphigoid gestationis: a case study. ( 25506008 )
2014
35
Pemphigoid gestationis with IgG autoantibodies to both the 120A kDa LAD-1 and the BP180 NC16a domain. ( 25547184 )
2014
36
Pemphigoid gestationis (herpes gestationis). ( 25404804 )
2014
37
Clinical and immunological profiles of 25 patients with pemphigoid gestationis. ( 25154546 )
2014
38
A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen. ( 23082779 )
2014
39
Intravenous Immunoglobulin Therapy for Persistent Pemphigoid Gestationis with Steroid Induced Iatrogenic Cushing's Syndrome. ( 25324672 )
2014
40
Pemphigoid gestationis. ( 24365006 )
2013
41
Pemphigoid gestationis: a retrospective study in southwest Iran. ( 23852847 )
2013
42
Pemphigoid gestationis. ( 24007701 )
2013
43
Pemphigoid gestationis after spontaneous expulsion of a massive complete hydatidiform mole. ( 24083041 )
2013
44
Dyshidrosiform presentation (pompholyx-like) of pemphigoid gestationis with intrauterine fetal death. ( 23432199 )
2013
45
Pemphigoid gestationis with postpartum flare-up: a case report. ( 24568052 )
2013
46
The utility of C4d immunohistochemistry on formalin-fixed paraffin-embedded tissue in the distinction of polymorphic eruption of pregnancy from pemphigoid gestationis. ( 24061402 )
2013
47
Suspicion of pulmonary embolism during treatment of pemphigoid gestationis. ( 24278048 )
2013
48
Pemphigoid gestationis. ( 22137226 )
2012
49
Emergency caesarean delivery of a parturient with undiagnosed pemphigoid gestationis. ( 22626993 )
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

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62 PubMed
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69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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