MCID: PMP002
MIFTS: 43

Pemphigoid Gestationis

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Pemphigoid Gestationis

MalaCards integrated aliases for Pemphigoid Gestationis:

Name: Pemphigoid Gestationis 12 54 60 15 17 74
Herpes Gestationis 12 54 56
Gestational Pemphigoid 54 60
Gestational Herpes 12

Classifications:

Orphanet: 60  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:14482
MeSH 45 D006559
NCIt 51 C85003
SNOMED-CT 69 86081009
ICD10 34 O26.4 O26.40
MESH via Orphanet 46 D006559
ICD10 via Orphanet 35 L12.8
UMLS via Orphanet 75 C0019343
Orphanet 60 ORPHA63275
UMLS 74 C0019343

Summaries for Pemphigoid Gestationis

NIH Rare Diseases : 54 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. Signs and 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. While PG usually goes away on its own within weeks to months after delivery, it has been reported to persist for years in some cases. 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. Treatment aims to relieve itching and prevent blister formation, and may involve the use of topical corticosteroids, oral corticosteroids, and/or oral antihistamines. The lowest effective dose of medication should be used in order to minimize the risk to the mother and fetus. The disorder may recur at a later time such as when menstruation resumes; with use of oral contraceptives; and/or during subsequent pregnancies.

MalaCards based summary : Pemphigoid Gestationis, also known as herpes gestationis, is related to pemphigus and lichen planus pemphigoides, 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 Allograft rejection and Hematopoietic cell lineage. Affiliated tissues include skin, placenta and testes, and related phenotypes are abnormal blistering of the skin and pruritus

Disease Ontology : 12 A vesiculobullous skin disease 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.

Wikipedia : 77 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 127)
# Related Disease Score Top Affiliating Genes
1 pemphigus 30.7 COL17A1 DST
2 lichen planus pemphigoides 30.4 COL17A1 DST
3 lichen planus 30.4 COL17A1 DST
4 linear iga disease 30.4 COL17A1 DST
5 cicatricial pemphigoid 30.3 COL17A1 DST
6 pemphigus vulgaris 30.0 DST HLA-DRB1
7 bullous pemphigoid 28.8 CCL11 COL17A1 DST HLA-DRB1 IL5 RNASE3
8 pemphigus gestationis 11.9
9 recessive dystrophic epidermolysis bullosa-generalized other 11.4
10 pruritic urticarial papules and plaques of pregnancy 10.4
11 dermatitis herpetiformis, familial 10.3
12 dermatitis 10.3
13 dermatitis herpetiformis 10.3
14 choriocarcinoma 10.3
15 neurotic excoriation 10.2 COL17A1 DST
16 pemphigus vulgaris, familial 10.2
17 hydatidiform mole, recurrent, 1 10.2
18 autoimmune disease 1 10.2
19 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.2
20 gestational trophoblastic neoplasm 10.2
21 epidermolysis bullosa 10.2 COL17A1 DST
22 epidermolysis bullosa simplex 10.2 COL17A1 DST
23 epidermolysis bullosa simplex with muscular dystrophy 10.2 COL17A1 DST
24 vesiculobullous skin disease 10.2 COL17A1 DST
25 autoimmune disease of skin and connective tissue 10.2 COL17A1 DST
26 epidermolysis bullosa acquisita 10.2 COL17A1 DST
27 lichen disease 10.2 COL17A1 DST
28 ecthyma 10.1 COL17A1 DST
29 epidermolysis bullosa junctionalis with pyloric atresia 10.1 COL17A1 DST
30 blepharoconjunctivitis 10.1 CCL11 RNASE3
31 subcorneal pustular dermatosis 10.1 COL17A1 DST
32 giant papillary conjunctivitis 10.1 CCL11 RNASE3
33 epidermolysis bullosa, junctional, non-herlitz type 10.1 COL17A1 DST
34 junctional epidermolysis bullosa 10.1 COL17A1 DST
35 pneumonia 10.1 CCL11 IL5
36 loeffler endocarditis 10.1 IL5 RNASE3
37 thrombocytopenia 10.1
38 erythema multiforme 10.1
39 intrahepatic cholestasis of pregnancy 10.1
40 gestational diabetes 10.1
41 conn's syndrome 10.1
42 graves' disease 10.1
43 hellp syndrome 10.1
44 cholestasis 10.1
45 facial hemiatrophy 10.1
46 chorioangioma 10.1
47 acute disseminated encephalomyelitis 10.1
48 pompholyx 10.1
49 pulmonary embolism 10.1
50 chronic meningitis 10.1 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:

60 33 (show all 6)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 abnormal blistering of the skin 60 33 hallmark (90%) Very frequent (99-80%) HP:0008066
2 pruritus 60 33 hallmark (90%) Very frequent (99-80%) HP:0000989
3 premature birth 60 33 hallmark (90%) Very frequent (99-80%) HP:0001622
4 skin vesicle 60 33 hallmark (90%) Very frequent (99-80%) HP:0200037
5 failure to thrive 60 33 frequent (33%) Frequent (79-30%) HP:0001508
6 intrauterine growth retardation 60 33 frequent (33%) Frequent (79-30%) HP:0001511

Symptoms:

12
  • pruritis

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 Unknown status NCT02874079 Not Applicable
2 Register of Autoimmune Bullous Dermatoses Completed NCT02899923

Search NIH Clinical Center for Pemphigoid Gestationis

Genetic Tests for Pemphigoid Gestationis

Anatomical Context for Pemphigoid Gestationis

MalaCards organs/tissues related to Pemphigoid Gestationis:

42
Skin, Placenta, Testes, T Cells

Publications for Pemphigoid Gestationis

Articles related to Pemphigoid Gestationis:

(show top 50) (show all 141)
# Title Authors Year
1
Pemphigoid Gestationis Mimicking Erythema Multiforme With Mucosal Involvement. ( 30732852 )
2019
2
Pemphigoid Gestationis. ( 30775676 )
2019
3
Pemphigoid Gestationis: A Case Presentation. ( 29486593 )
2018
4
Pemphigoid gestationis successfully treated with intravenous immunoglobulin. ( 29627782 )
2018
5
Pemphigoid gestationis with lethal fetal malformation and postpartum persistence. ( 29873913 )
2018
6
Anhydramnios in Patients With Pemphigoid Gestationis. ( 29365022 )
2018
7
Pemphigoid gestationis mit letaler fAPtaler Fehlbildung und postpartaler Persistenz. ( 29873912 )
2018
8
Pemphigoid gestationis: a case series and review of the literature. ( 29620424 )
2018
9
Pemphigoid gestationis. ( 29988643 )
2018
10
Pemphigoid gestationis and intravenous immunoglobulin therapy. ( 30175219 )
2018
11
Specific dermatoses of pregnancy other than pemphigoid gestationis. ( 30375214 )
2018
12
Pemphigoid gestationis: Clinical and histologic features of twenty-three patients. ( 28560301 )
2017
13
Early flare-up of severe Herpes gestationis (Pemphigoid gestationis) and successfull prolonged treatment: A case report. ( 28399702 )
2017
14
Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder. ( 28524368 )
2017
15
Pemphigoid gestationis: current perspectives. ( 29184427 )
2017
16
Pemphigoid Gestationis ( 29261895 )
2017
17
Sensitivity and specificity of BP180 NC16A enzyme-linked immunosorbent assay for the diagnosis of pemphigoid gestationis. ( 28212762 )
2017
18
Image Gallery: Pemphigoid gestationis mimicking a gyrate erythema. ( 28418130 )
2017
19
Concomitant pemphigus vulgaris and pemphigoid gestationis: a case report and review of the literature. ( 26990471 )
2016
20
Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis. ( 27265076 )
2016
21
Pemphigoid gestationis: a successful preventive treatment by rituximab. ( 27606493 )
2016
22
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
23
New Technology Identifies Pemphigoid Gestationis by Detection of Anti-BP180 IgG, but Simultaneous Detection of Anti-BP230 IgG does not Improve Diagnosis. ( 27174635 )
2016
24
Pregnancy with Pemphigoid Gestationis: A Rare Entity. ( 27630915 )
2016
25
Retrospective analysis of pemphigoid gestationis in 32 Saudi patients - Clinicopathological features and a literature review. ( 27175516 )
2016
26
Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis. ( 27416093 )
2016
27
Dermatological Diseases Associated with Pregnancy: Pemphigoid Gestationis, Polymorphic Eruption of Pregnancy, Intrahepatic Cholestasis of Pregnancy, and Atopic Eruption of Pregnancy. ( 26609305 )
2015
28
Pemphigoid gestationis. ( 25581583 )
2015
29
What is your diagnosis? pemphigoid gestationis (herpes gestationis). ( 26057509 )
2015
30
Positive clinical outcome with IVIg as monotherapy in recurrent pemphigoid gestationis. ( 25765353 )
2015
31
Pemphigoid gestationis revealing a denial of pregnancy. ( 26299950 )
2015
32
A severe and prolonged case of pemphigoid gestationis successfully treated with combination therapies. ( 26521896 )
2015
33
Pemphigoid Gestationis Complicating an Egg Donation Pregnancy. ( 26608214 )
2015
34
Pemphigoid Gestationis: A Case Report and Review of Management. ( 26267738 )
2015
35
Pemphigoid gestationis with prepartum flare. ( 25039537 )
2014
36
Pemphigoid gestationis in a third trimester pregnancy. ( 25431712 )
2014
37
Successfully treated case of pemphigoid gestationis with post-partum intravenous and oral steroids. ( 25404796 )
2014
38
Case for diagnosis. Pemphigoid gestationis. ( 24626668 )
2014
39
Recognizing presentations of pemphigoid gestationis: a case study. ( 25506008 )
2014
40
Pemphigoid gestationis with IgG autoantibodies to both the 120A kDa LAD-1 and the BP180 NC16a domain. ( 25547184 )
2014
41
Pemphigoid gestationis (herpes gestationis). ( 25404804 )
2014
42
Clinical and immunological profiles of 25 patients with pemphigoid gestationis. ( 25154546 )
2014
43
A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen. ( 23082779 )
2014
44
Intravenous Immunoglobulin Therapy for Persistent Pemphigoid Gestationis with Steroid Induced Iatrogenic Cushing's Syndrome. ( 25324672 )
2014
45
Pemphigoid gestationis. ( 24365006 )
2013
46
Pemphigoid gestationis: a retrospective study in southwest Iran. ( 23852847 )
2013
47
Pemphigoid gestationis. ( 24007701 )
2013
48
Pemphigoid gestationis after spontaneous expulsion of a massive complete hydatidiform mole. ( 24083041 )
2013
49
Dyshidrosiform presentation (pompholyx-like) of pemphigoid gestationis with intrauterine fetal death. ( 23432199 )
2013
50
Pemphigoid gestationis with postpartum flare-up: a case report. ( 24568052 )
2013

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 9.13 CCL11 HLA-DRB1 IL5
2 hemidesmosome assembly GO:0031581 8.62 COL17A1 DST

Sources for Pemphigoid Gestationis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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