MCID: PMP002
MIFTS: 35

Pemphigoid Gestationis malady

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Pemphigoid Gestationis

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Sources:
31ICD10 via Orphanet, 40MESH via Orphanet, 48NIH Rare Diseases, 54Orphanet, 68UMLS, 69UMLS via Orphanet
See all MalaCards sources

Aliases & Descriptions for Pemphigoid Gestationis:

Name: Pemphigoid Gestationis 48 54 68
Gestational Pemphigoid 48 54
 
Herpes Gestationis 48

Classifications:

Orphanet: 54 
Rare skin diseases


External Ids:

Orphanet54 ORPHA63275
ICD10 via Orphanet31 L12.8
UMLS via Orphanet69 C0019343
MESH via Orphanet40 D006559

Summaries for Pemphigoid Gestationis

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NIH Rare Diseases:48 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 pompholyx and herpes gestationis, and has symptoms including morning sickness, Array and Array. An important gene associated with Pemphigoid Gestationis is COL17A1 (Collagen Type XVII Alpha 1 Chain), and among its related pathways are Lung fibrosis and IL4-mediated signaling events. Affiliated tissues include skin, placenta and t cells.

Wikipedia:71 Gestational pemphigoid or pemphigoid gestationis (PG) is a dermatosis of pregnancy, being an autoimmune... more...

Related Diseases for Pemphigoid Gestationis

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

(show top 50)    (show all 91)
idRelated DiseaseScoreTop Affiliating Genes
1pompholyx29.9COL17A1, DST
2herpes gestationis12.0
3linear hamartoma syndrome10.2COL17A1, DST
4lipoblastoma10.2COL17A1, DST
5dyskinesia of esophagus10.2COL17A1, DST
6nemaline myopathy 910.2COL17A1, DST
7epidermolytic acanthoma10.2COL17A1, DST
8atypical autism10.2COL17A1, DST
9bilirubin metabolic disorder10.2COL17A1, DST
10myelitis10.2COL17A1, DST
11nephrosclerosis10.1COL17A1, DST
12oral cavity cancer10.1COL17A1, DST
13nephrotic syndrome, type 210.1COL17A1, DST
14bullous pemphigoid10.1
15peripheral nervous system neoplasm10.1COL17A1, DST
16herpes zoster10.1COL17A1, DST
17ureteral lymphoma10.1COL17A1, DST
18lin-gettig syndrome10.1COL17A1, HLA-DRB1
19intraocular retinoblastoma10.1COL17A1, DST
20mast-cell leukemia10.1COL17A1, DST
21peripheral t-cell lymphoma10.1DST, HLA-DRB1
22gilbert syndrome10.1COL17A1, DST
23pol iii-related leukodystrophies10.1DST, HLA-DRB1
24natural killer cell leukemia10.0CCL11, IL5
25thelaziasis10.0CCL11, IL5
26lymphoblastic leukemia10.0CCL11, IL5
27spastic diplegia10.0CCL11, IL5
28latent syphilis10.0CCL11, IL5
29necrotizing gastritis10.0CCL11, IL5
30disordered steroidogenesis due to cytochrome p450 oxidoreductase10.0CCL11, IL5
31cerebral primitive neuroectodermal tumor10.0CCL11, IL5
32apricot allergy10.0CCL11, IL5
33trachea leiomyoma10.0CCL11, IL5
34visceral steatosis10.0CCL11, IL5
35cutaneous fibrous histiocytoma10.0CCL11, IL5
36lymphocytic gastritis10.0CCL11, IL5
37intravascular angioleiomyoma10.0CCL11, IL5
38ethmoid sinusitis10.0CCL11, IL5
39childhood malignant mesenchymoma10.0CCL11, IL5
40inflamed seborrheic keratosis10.0CCL11, IL5
41hydrophthalmos10.0CCL11, IL5
42eyelid neoplasm10.0CCL11, IL5
43patellar tendinitis10.0CCL11, IL5
44acquired polycythemia10.0CCL11, IL5
45tracheitis10.0CCL11, IL5
46albinism10.0HLA-DRB1, IL5
47thyroid hyalinizing trabecular adenoma10.0CCL11, IL5
48xanthogranulomatous cholecystitis10.0CCL11, IL5
49colon adenocarcinoma10.0CCL11, IL5
50conjunctival concretion10.0CCL11, IL5

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



Diseases related to pemphigoid gestationis

Symptoms & Phenotypes for Pemphigoid Gestationis

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Human phenotypes related to Pemphigoid Gestationis:

 54 64 (show all 6)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 pruritus64 54 Very frequent (99-80%) HP:0000989
2 failure to thrive64 54 Frequent (79-30%) HP:0001508
3 intrauterine growth retardation64 54 Frequent (79-30%) HP:0001511
4 premature birth64 54 Very frequent (99-80%) HP:0001622
5 abnormal blistering of the skin64 54 Very frequent (99-80%) HP:0008066
6 skin vesicle64 54 Very frequent (99-80%) HP:0200037

UMLS symptoms related to Pemphigoid Gestationis:


morning sickness

Drugs & Therapeutics for Pemphigoid Gestationis

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Interventional clinical trials:

idNameStatusNCT IDPhase
1Genetic Susceptibility and Influence of the Microbiomae in Bullous PemphigoidRecruitingNCT02874079

Search NIH Clinical Center for Pemphigoid Gestationis

Genetic Tests for Pemphigoid Gestationis

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Anatomical Context for Pemphigoid Gestationis

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MalaCards organs/tissues related to Pemphigoid Gestationis:

36
Skin, Placenta, T cells, Testes

Publications for Pemphigoid Gestationis

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Articles related to Pemphigoid Gestationis:

(show top 50)    (show all 117)
idTitleAuthorsYear
1
Retrospective analysis of pemphigoid gestationis in 32 Saudi patients - Clinicopathological features and a literature review. (27175516)
2016
2
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
3
Pregnancy with Pemphigoid Gestationis: A Rare Entity. (27630915)
2016
4
Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis. (27416093)
2016
5
Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis. (27265076)
2016
6
New Technology Identifies Pemphigoid Gestationis by Detection of Anti-BP180 IgG, but Simultaneous Detection of Anti-BP230 IgG does not Improve Diagnosis. (27174635)
2016
7
Pemphigoid gestationis: a successful preventive treatment by rituximab. (27606493)
2016
8
Concomitant pemphigus vulgaris and pemphigoid gestationis: a case report and review of the literature. (26990471)
2016
9
Pemphigoid gestationis. (25581583)
2015
10
Dermatological Diseases Associated with Pregnancy: Pemphigoid Gestationis, Polymorphic Eruption of Pregnancy, Intrahepatic Cholestasis of Pregnancy, and Atopic Eruption of Pregnancy. (26609305)
2015
11
What is your diagnosis? pemphigoid gestationis (herpes gestationis). (26057509)
2015
12
Pemphigoid Gestationis: A Case Report and Review of Management. (26267738)
2015
13
Pemphigoid Gestationis Complicating an Egg Donation Pregnancy. (26608214)
2015
14
Pemphigoid gestationis revealing a denial of pregnancy. (26299950)
2015
15
Positive clinical outcome with IVIg as monotherapy in recurrent pemphigoid gestationis. (25765353)
2015
16
A severe and prolonged case of pemphigoid gestationis successfully treated with combination therapies. (26521896)
2015
17
Pemphigoid gestationis with IgG autoantibodies to both the 120A kDa LAD-1 and the BP180 NC16a domain. (25547184)
2014
18
Recognizing presentations of pemphigoid gestationis: a case study. (25506008)
2014
19
Pemphigoid gestationis (herpes gestationis). (25404804)
2014
20
Pemphigoid gestationis in a third trimester pregnancy. (25431712)
2014
21
Clinical and immunological profiles of 25 patients with pemphigoid gestationis. (25154546)
2014
22
Successfully treated case of pemphigoid gestationis with post-partum intravenous and oral steroids. (25404796)
2014
23
A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen. (23082779)
2014
24
Pemphigoid gestationis with prepartum flare. (25039537)
2014
25
Intravenous Immunoglobulin Therapy for Persistent Pemphigoid Gestationis with Steroid Induced Iatrogenic Cushing's Syndrome. (25324672)
2014
26
Case for diagnosis. Pemphigoid gestationis. (24626668)
2014
27
Dyshidrosiform presentation (pompholyx-like) of pemphigoid gestationis with intrauterine fetal death. (23432199)
2013
28
Suspicion of pulmonary embolism during treatment of pemphigoid gestationis. (24278048)
2013
29
The utility of C4d immunohistochemistry on formalin-fixed paraffin-embedded tissue in the distinction of polymorphic eruption of pregnancy from pemphigoid gestationis. (24061402)
2013
30
Pemphigoid gestationis: a retrospective study in southwest Iran. (23852847)
2013
31
Pemphigoid gestationis. (24007701)
2013
32
Pemphigoid gestationis after spontaneous expulsion of a massive complete hydatidiform mole. (24083041)
2013
33
Pemphigoid gestationis with postpartum flare-up: a case report. (24568052)
2013
34
Pemphigoid gestationis. (24365006)
2013
35
Successful treatment of a severe persistent case of pemphigoid gestationis with antepartum and postpartum intravenous immunoglobulin followed by azathioprine. (22309336)
2012
36
Emergency caesarean delivery of a parturient with undiagnosed pemphigoid gestationis. (22626993)
2012
37
Pemphigoid gestationis in a female with progressive facial hemiatrophy: microchimerism as a speculative shared background is disputable. (22535610)
2012
38
Postpartum pemphigoid gestationis. (22615518)
2012
39
Pemphigoid gestationis. (22137226)
2012
40
Pemphigoid gestationis with a complete hydatidiform mole. (21950371)
2012
41
Glucocorticosteroid-resistant pemphigoid gestationis: successful treatment with adjuvant immunoadsorption. (22379622)
2012
42
Refractory pemphigoid gestationis postpartum. (22360419)
2012
43
Pemphigoid gestationis: pathogenesis and clinical features. (21605810)
2011
44
Pemphigoid gestationis. (22715180)
2011
45
Pemphigoid gestationis: current management. (21925007)
2011
46
Successful immunoapheresis of bullous autoimmune diseases: pemphigus vulgaris and pemphigoid gestationis. (20812992)
2011
47
Pemphigoid gestationis: a case report and review of the literature. (21877502)
2011
48
Pemphigoid gestationis: cutaneous manifestation of impaired fetal allograft tolerance. (20527151)
2010
49
Pemphigoid gestationis. (21163161)
2010
50
Antepartum intravenous immunoglobulin therapy in refractory pemphigoid gestationis: case report and literature review. (20642990)
2010

Variations for Pemphigoid Gestationis

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Expression for genes affiliated with Pemphigoid Gestationis

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Search GEO for disease gene expression data for Pemphigoid Gestationis.

Pathways for genes affiliated with Pemphigoid Gestationis

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GO Terms for genes affiliated with Pemphigoid Gestationis

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

idNameGO IDScoreTop Affiliating Genes
1basement membraneGO:00056049.7COL17A1, DST
2hemidesmosomeGO:00300569.5COL17A1, DST

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

idNameGO IDScoreTop Affiliating Genes
1immune responseGO:00069558.5CCL11, HLA-DRB1, IL5

Sources for Pemphigoid Gestationis

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet