MCID: PMP002
MIFTS: 34

Pemphigoid Gestationis malady

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Pemphigoid Gestationis

About this section
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

About this section
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 herpes gestationis and lichen sclerosus, and has symptoms including premature birth, urticaria and Array. An important gene associated with Pemphigoid Gestationis is COL17A1 (Collagen Type XVII Alpha 1 Chain), and among its related pathways are Alpha6-Beta4 Integrin Signaling Pathway 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

About this section

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

(show top 50)    (show all 86)
idRelated DiseaseScoreTop Affiliating Genes
1herpes gestationis12.0
2lichen sclerosus10.3COL17A1, DST
3submandibular gland cancer10.3COL17A1, DST
4microcephaly, holoprosencephaly, and intrauterine growth retardation10.3COL17A1, DST
5gilbert syndrome10.2COL17A1, DST
6impetigo10.2COL17A1, DST
7malignant histiocytosis10.2COL17A1, DST
8puerperal pulmonary embolism10.2COL17A1, DST
9tinea cruris10.2COL17A1, DST
10demyelinating disease10.2COL17A1, DST
11sporotrichosis10.2COL17A1, DST
12quinquaud's decalvans folliculitis10.2COL17A1, HLA-DRB1
13bullous pemphigoid10.1
14familial retinoblastoma10.1COL17A1, DST
15geniculate herpes zoster10.1COL17A1, DST
16peri-anal fistula10.1DST, HLA-DRB1
17blood group incompatibility10.1COL17A1, DST
18borst-jadassohn intraepidermal carcinoma10.1CCL11, HLA-DRB1
19iridocyclitis10.0CCL11, HLA-DRB1
20skin atrophy10.0COL17A1, DST
21solitary cyst of breast10.0CCL11, IL5
22cholesteatoma of middle ear9.9CCL11, IL5
23fungal gastritis9.9CCL11, IL5
24vibrio vulnificus infection9.9CCL11, IL5
25pruritic urticarial papules and plaques of pregnancy9.9
26pemphigus vulgaris9.9
27pemphigus9.9
28antley-bixler syndrome with genital anomalies and disordered steroidogenesis9.9CCL11, IL5
29benign mammary dysplasia9.9CCL11, IL5
30dioctophymiasis9.9CCL11, IL5
31epididymis adenocarcinoma9.9CCL11, IL5
32bacterial gastritis9.9CCL11, IL5
33apple allergy9.9CCL11, IL5
34cervix melanoma9.9CCL11, IL5
35central nervous system origin vertigo9.9CCL11, IL5
36pituitary hypoplasia9.9CCL11, IL5
37dacryoadenitis9.9CCL11, IL5
38gait apraxia9.9CCL11, IL5
39buphthalmos9.9CCL11, IL5
40melanoacanthoma9.9CCL11, IL5
41vaginitis9.9CCL11, IL5
42geotrichosis9.9CCL11, IL5
43dentin caries9.9CCL11, IL5
44small non-cleaved cell lymphoma9.9COL17A1, DST
45sarcoid meningitis9.9CCL11, IL5
46biliary tract disease9.9CCL11, IL5
47loeffler syndrome9.9CCL11, IL5
48purpura fulminans9.8HLA-DRB1, IL5
49synostosis9.8CCL11, IL5
50exophthalmos9.8CCL11, IL5

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



Diseases related to pemphigoid gestationis

Symptoms & Phenotypes for Pemphigoid Gestationis

About this section

Human phenotypes related to Pemphigoid Gestationis:

 64 54 (show all 7)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 premature birth64 54 hallmark (90%) Very frequent (99-80%) HP:0001622
2 urticaria64 occasional (7.5%) HP:0001025
3 pruritus54 Very frequent (99-80%)
4 failure to thrive54 Frequent (79-30%)
5 intrauterine growth retardation54 Frequent (79-30%)
6 abnormal blistering of the skin54 Very frequent (99-80%)
7 skin vesicle54 Very frequent (99-80%)

UMLS symptoms related to Pemphigoid Gestationis:


hyperemesis gravidarum, morning sickness, coma blister

Drugs & Therapeutics for Pemphigoid Gestationis

About this section

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

About this section

Anatomical Context for Pemphigoid Gestationis

About this section

MalaCards organs/tissues related to Pemphigoid Gestationis:

36
Skin, Placenta, T cells, Testes

Publications for Pemphigoid Gestationis

About this section

Articles related to Pemphigoid Gestationis:

(show top 50)    (show all 117)
idTitleAuthorsYear
1
Pemphigoid gestationis: a successful preventive treatment by rituximab. (27606493)
2016
2
Concomitant pemphigus vulgaris and pemphigoid gestationis: a case report and review of the literature. (26990471)
2016
3
Lichen planus pemphigoides associated with pregnancy mimicking pemphigoid gestationis. (27416093)
2016
4
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
5
New Technology Identifies Pemphigoid Gestationis by Detection of Anti-BP180 IgG, but Simultaneous Detection of Anti-BP230 IgG does not Improve Diagnosis. (27174635)
2016
6
Pregnancy with Pemphigoid Gestationis: A Rare Entity. (27630915)
2016
7
Retrospective analysis of pemphigoid gestationis in 32 Saudi patients - Clinicopathological features and a literature review. (27175516)
2016
8
Pemphigoid gestationis: Toward a better understanding of the etiopathogenesis. (27265076)
2016
9
Pemphigoid gestationis revealing a denial of pregnancy. (26299950)
2015
10
Pemphigoid gestationis. (25581583)
2015
11
Positive clinical outcome with IVIg as monotherapy in recurrent pemphigoid gestationis. (25765353)
2015
12
What is your diagnosis? pemphigoid gestationis (herpes gestationis). (26057509)
2015
13
Pemphigoid Gestationis: A Case Report and Review of Management. (26267738)
2015
14
Pemphigoid Gestationis Complicating an Egg Donation Pregnancy. (26608214)
2015
15
Dermatological Diseases Associated with Pregnancy: Pemphigoid Gestationis, Polymorphic Eruption of Pregnancy, Intrahepatic Cholestasis of Pregnancy, and Atopic Eruption of Pregnancy. (26609305)
2015
16
A severe and prolonged case of pemphigoid gestationis successfully treated with combination therapies. (26521896)
2015
17
Pemphigoid gestationis with prepartum flare. (25039537)
2014
18
Pemphigoid gestationis (herpes gestationis). (25404804)
2014
19
Successfully treated case of pemphigoid gestationis with post-partum intravenous and oral steroids. (25404796)
2014
20
Clinical and immunological profiles of 25 patients with pemphigoid gestationis. (25154546)
2014
21
Pemphigoid gestationis in a third trimester pregnancy. (25431712)
2014
22
Case for diagnosis. Pemphigoid gestationis. (24626668)
2014
23
A case of pemphigoid gestationis with concurrent IgG antibodies to BP180, BP230 and type VII collagen. (23082779)
2014
24
Recognizing presentations of pemphigoid gestationis: a case study. (25506008)
2014
25
Intravenous Immunoglobulin Therapy for Persistent Pemphigoid Gestationis with Steroid Induced Iatrogenic Cushing's Syndrome. (25324672)
2014
26
Pemphigoid gestationis with IgG autoantibodies to both the 120A kDa LAD-1 and the BP180 NC16a domain. (25547184)
2014
27
The utility of C4d immunohistochemistry on formalin-fixed paraffin-embedded tissue in the distinction of polymorphic eruption of pregnancy from pemphigoid gestationis. (24061402)
2013
28
Pemphigoid gestationis. (24365006)
2013
29
Pemphigoid gestationis after spontaneous expulsion of a massive complete hydatidiform mole. (24083041)
2013
30
Pemphigoid gestationis with postpartum flare-up: a case report. (24568052)
2013
31
Suspicion of pulmonary embolism during treatment of pemphigoid gestationis. (24278048)
2013
32
Dyshidrosiform presentation (pompholyx-like) of pemphigoid gestationis with intrauterine fetal death. (23432199)
2013
33
Pemphigoid gestationis. (24007701)
2013
34
Pemphigoid gestationis: a retrospective study in southwest Iran. (23852847)
2013
35
Pemphigoid gestationis in a female with progressive facial hemiatrophy: microchimerism as a speculative shared background is disputable. (22535610)
2012
36
Glucocorticosteroid-resistant pemphigoid gestationis: successful treatment with adjuvant immunoadsorption. (22379622)
2012
37
Successful treatment of a severe persistent case of pemphigoid gestationis with antepartum and postpartum intravenous immunoglobulin followed by azathioprine. (22309336)
2012
38
Emergency caesarean delivery of a parturient with undiagnosed pemphigoid gestationis. (22626993)
2012
39
Refractory pemphigoid gestationis postpartum. (22360419)
2012
40
Postpartum pemphigoid gestationis. (22615518)
2012
41
Pemphigoid gestationis with a complete hydatidiform mole. (21950371)
2012
42
Pemphigoid gestationis. (22137226)
2012
43
Pemphigoid gestationis. (22715180)
2011
44
Pemphigoid gestationis: current management. (21925007)
2011
45
Pemphigoid gestationis: pathogenesis and clinical features. (21605810)
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
No evidence for fetal microchimerism in the skin of patients with pemphigoid gestationis. (19840915)
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

About this section

Expression for genes affiliated with Pemphigoid Gestationis

About this section
Search GEO for disease gene expression data for Pemphigoid Gestationis.

Pathways for genes affiliated with Pemphigoid Gestationis

About this section

GO Terms for genes affiliated with Pemphigoid Gestationis

About this section

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

Sources for Pemphigoid Gestationis

About this section
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