MCID: PMP002
MIFTS: 38

Pemphigoid Gestationis

Categories: Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for Pemphigoid Gestationis

MalaCards integrated aliases for Pemphigoid Gestationis:

Name: Pemphigoid Gestationis 12 20 58 15 17 70
Herpes Gestationis 12 20 54 32
Gestational Pemphigoid 20 58
Gestational Herpes 12

Classifications:

Orphanet: 58  
Rare skin diseases


External Ids:

Disease Ontology 12 DOID:14482
MeSH 44 D006559
NCIt 50 C85003
SNOMED-CT 67 86081009
ICD10 32 O26.4
MESH via Orphanet 45 D006559
ICD10 via Orphanet 33 L12.8
UMLS via Orphanet 71 C0019343
Orphanet 58 ORPHA63275
UMLS 70 C0019343

Summaries for Pemphigoid Gestationis

GARD : 20 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 pemphigoid and dermatitis herpetiformis, 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 Th2 Differentiation Pathway. Affiliated tissues include skin, placenta and ovary, and related phenotypes are premature birth and pruritus

Disease Ontology : 12 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.

Wikipedia : 73 Gestational pemphigoid (GP) is an autoimmune blistering skin disease of pregnancy, typically occurring... 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 195)
# Related Disease Score Top Affiliating Genes
1 pemphigoid 31.3 IL5 HLA-DRB1 DST COL17A1 CCL11
2 dermatitis herpetiformis 30.9 IL5 CCL11
3 lichen planus pemphigoides 30.7 DST COL17A1
4 linear iga disease 30.5 DST COL17A1
5 pompholyx 30.5 DST COL17A1
6 cicatricial pemphigoid 30.4 HLA-DRB1 DST COL17A1
7 epidermolysis bullosa acquisita 30.2 HLA-DRB1 DST COL17A1
8 hypereosinophilic syndrome 30.0 RNASE3 IL5 CCL11
9 autoimmune disease 29.8 IL5 HLA-DRB1 DSG3 DSG1 COL17A1
10 lichen planus 29.6 DST DSG3 DSG1 COL17A1
11 dermatitis 29.6 RNASE3 IL5 DSG1 CCL11
12 pemphigus 29.5 HLA-DRB1 DST DSG3 DSG1 COL17A1
13 pemphigus foliaceus 29.5 HLA-DRB1 DST DSG3 DSG1
14 celiac disease 1 29.4 TNFRSF25 TNFRSF10A IL5 HLA-DRB1
15 pemphigus vulgaris, familial 29.3 HLA-DRB1 DST DSG3 DSG1 COL17A1
16 bullous pemphigoid 29.2 RNASE3 IL5 HLA-DRB1 DST DSG3 DSG1
17 skin disease 29.1 RNASE3 IL5 DST DSG3 DSG1 COL17A1
18 paraneoplastic pemphigus 29.0 HLA-DRB1 DST DSG3 DSG1 COL17A1
19 bullous skin disease 28.6 TNFRSF25 IL5 DST DSG3 DSG1 COL17A1
20 pemphigus gestationis 11.5
21 recessive dystrophic epidermolysis bullosa-generalized other 11.2
22 dermatitis herpetiformis, familial 10.5
23 exanthem 10.4
24 pruritic urticarial papules and plaques of pregnancy 10.4
25 neurotic excoriation 10.3 DST COL17A1
26 vulvar dystrophy 10.3 DST COL17A1
27 mite infestation 10.2 IL5 COL17A1
28 loiasis 10.2 IL5 CCL11
29 eosinophilic gastritis 10.2 IL5 CCL11
30 epidermolysis bullosa junctionalis with pyloric atresia 10.2 DST COL17A1
31 trichinosis 10.2 IL5 CCL11
32 retroperitoneal fibrosis 10.2 HLA-DRB1 CCL11
33 epidermolysis bullosa simplex with muscular dystrophy 10.2 DST COL17A1
34 chronic meningitis 10.2 IL5 CCL11
35 angiostrongyliasis 10.2 IL5 CCL11
36 strongyloidiasis 10.2 IL5 CCL11
37 eosinophilic meningitis 10.2 IL5 CCL11
38 acute asthma 10.2 RNASE3 IL5
39 adhesive otitis media 10.2 RNASE3 CCL11
40 loeffler endocarditis 10.2 RNASE3 IL5
41 eustachian tube disease 10.2 RNASE3 CCL11
42 atopic keratoconjunctivitis 10.2 RNASE3 IL5
43 kindler syndrome 10.2 DST COL17A1
44 eosinophilia-myalgia syndrome 10.2 IL5 HLA-DRB1
45 cough variant asthma 10.2 RNASE3 IL5
46 ecthyma 10.2 DST DSG1
47 disseminated eosinophilic collagen disease 10.2 RNASE3 IL5
48 kimura disease 10.2 RNASE3 IL5
49 folliculitis 10.2 IL5 CCL11
50 timothy grass allergy 10.2 RNASE3 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:

58 31 (show all 6)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 premature birth 58 31 hallmark (90%) Very frequent (99-80%) HP:0001622
2 pruritus 58 31 hallmark (90%) Very frequent (99-80%) HP:0000989
3 skin vesicle 58 31 hallmark (90%) Very frequent (99-80%) HP:0200037
4 abnormal blistering of the skin 58 31 hallmark (90%) Very frequent (99-80%) HP:0008066
5 failure to thrive 58 31 frequent (33%) Frequent (79-30%) HP:0001508
6 intrauterine growth retardation 58 31 frequent (33%) Frequent (79-30%) HP:0001511

Symptoms:

12
  • pruritis

UMLS symptoms related to Pemphigoid Gestationis:


morning sickness

Drugs & Therapeutics for Pemphigoid Gestationis

Search Clinical Trials , NIH Clinical Center for Pemphigoid Gestationis

Genetic Tests for Pemphigoid Gestationis

Anatomical Context for Pemphigoid Gestationis

MalaCards organs/tissues related to Pemphigoid Gestationis:

40
Skin, Placenta, Ovary, T Cells

Publications for Pemphigoid Gestationis

Articles related to Pemphigoid Gestationis:

(show top 50) (show all 600)
# Title Authors PMID Year
1
Pemphigoid gestationis: clinical and laboratory evaluation. 54 61
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. 61 54
17875879 2007
3
Molecular mapping of the major epitopes of BP180 recognized by herpes gestationis autoantibodies. 61 54
10479533 1999
4
Acquired skin disease of hemidesmosomes. 61 54
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. 61 54
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. 54 61
8874743 1996
7
Cicatricial pemphigoid autoantibodies react with multiple sites on the BP180 extracellular domain. 61 54
8592065 1996
8
Molecular mapping of a pathogenically relevant BP180 epitope associated with experimentally induced murine bullous pemphigoid. 61 54
7594563 1995
9
Serum eosinophil cationic protein (ECP) in bullous pemphigoid. 54 61
7751092 1995
10
Development of an ELISA to detect anti-BP180 autoantibodies in bullous pemphigoid and herpes gestationis. 61 54
7516396 1994
11
[Herpes gestationis: immunologic and immunogenetic aspects]. 61 54
8113038 1993
12
Bullous pemphigoid and herpes gestationis autoantibodies recognize a common non-collagenous site on the BP180 ectodomain. 61 54
8228259 1993
13
A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. 54 61
7693763 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. 61 54
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. 54 61
1698819 1990
16
Subepidermal Autoimmune Blistering Dermatoses: Clinical Features and Diagnosis. 61
33684496 2021
17
Pemphigoid gestationis: a rare pregnancy dermatosis treated with a combination of IVIg and rituximab. 61
33727302 2021
18
Flame Figures in Linear Immunoglobulin A Bullous Dermatosis Secondary to Parvovirus B19 Infection. 61
32472771 2021
19
Pemphigoid Gestationis-A Rare Pregnancy Dermatoses. 61
33417630 2020
20
A Rare Case of Recurrent Postpartum Pemphigoid Gestationis with Fetal Complications and Skip Pregnancy. 61
33344355 2020
21
A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita. 61
32180161 2020
22
Pemphigoid Gestationis. 61
32846065 2020
23
Pemphigoid gestationis. 61
31506200 2020
24
[Pemphigoid gestationis after egg donation : A case report]. 61
31950210 2020
25
A Systematic Review of Treatment Options and Clinical Outcomes in Pemphigoid Gestationis. 61
33330568 2020
26
Treatment of Autoimmune Bullous Diseases During Pregnancy and Lactation: A Review Focusing on Pemphigus and Pemphigoid Gestationis. 61
33117178 2020
27
Dyshidrosiform Bullous Pemphigoid: Case Reports and Review. 61
32064205 2020
28
Postpartum pruritic urticarial papules and plaques of pregnancy with blister formation resembling herpes gestationis. 61
31903969 2019
29
Pemphigoid Gestationis Mimicking Erythema Multiforme With Mucosal Involvement. 61
30732852 2019
30
Review of autoimmune blistering diseases: the Pemphigoid diseases. 61
31087464 2019
31
Specific dermatoses of pregnancy other than pemphigoid gestationis. 61
30375214 2019
32
Autoimmune bullous diseases during pregnancy: insight into pathogenetic mechanisms and clinical features. 61
30375213 2019
33
Successful treatment of one case of pemphigoid gestationis complicating with polycystic ovary syndrome. 61
30963687 2019
34
Pemphigoid Gestationis. 61
30775676 2019
35
Lichen Planus Pemphigoides: From Lichenoid Inflammation to Autoantibody-Mediated Blistering. 61
31312198 2019
36
Targetoid form of polymorphic eruption of pregnancy: A case report. 61
31662850 2019
37
Pemphigoid gestationis: a case series and review of the literature. 61
29620424 2018
38
Pemphigoid gestationis and intravenous immunoglobulin therapy. 61
30175219 2018
39
Pemphigoid gestationis. 61
29988643 2018
40
Pemphigoid Gestationis: A Case Presentation. 61
29486593 2018
41
Pruritus in Pregnancy and Its Management. 61
29929597 2018
42
Treatment of Autoimmune Bullous Disorders in Pregnancy. 61
29392620 2018
43
Pemphigoid gestationis with lethal fetal malformation and postpartum persistence. 61
29873913 2018
44
Pemphigoid gestationis mit letaler fötaler Fehlbildung und postpartaler Persistenz. 61
29873912 2018
45
A subset of patients with pemphigoid (herpes) gestationis has serological evidence of celiac disease. 61
29473148 2018
46
Severe pemphigoid gestationis associated with acute disseminated encephalomyelitis in the setting of a systemic disorder. 61
28524368 2018
47
Case of epidermolysis bullosa acquisita with concomitant anti-laminin-332 antibodies. 61
29205468 2018
48
Anhydramnios in Patients With Pemphigoid Gestationis. 61
29365022 2018
49
Pemphigoid gestationis successfully treated with intravenous immunoglobulin. 61
29627782 2018
50
Urticarial Lesions in a Pregnant Woman. 61
29782306 2018

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
11.67 RNASE3 IL5 HLA-DRB1 CCL11
2 10.82 IL5 CCL11
3 10.42 IL5 HLA-DRB1

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 cell-cell junction GO:0005911 9.33 DSG3 DSG1 COL17A1
2 cornified envelope GO:0001533 9.26 DSG3 DSG1
3 desmosome GO:0030057 8.96 DSG3 DSG1
4 hemidesmosome GO:0030056 8.62 DST COL17A1

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

# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 9.46 DST DSG3 DSG1 CCL11
2 cytoskeleton organization GO:0007010 9.13 HLA-DRB1 DST CCL11
3 hemidesmosome assembly GO:0031581 8.62 DST COL17A1

Sources for Pemphigoid Gestationis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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