MCID: PMP002
MIFTS: 35

Pemphigoid Gestationis malady

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Pemphigoid Gestationis

Aliases & Descriptions for Pemphigoid Gestationis:

Name: Pemphigoid Gestationis 50 56 69
Gestational Pemphigoid 50 56
Herpes Gestationis 50

Classifications:

Orphanet: 56  
Rare skin diseases


External Ids:

Orphanet 56 ORPHA63275
ICD10 via Orphanet 34 L12.8
UMLS via Orphanet 70 C0019343
MESH via Orphanet 43 D006559

Summaries for Pemphigoid Gestationis

NIH Rare Diseases : 50 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 pruritus, failure to thrive and abnormal blistering of the skin. 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.

Wikipedia : 71 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 91)
id Related Disease Score Top Affiliating Genes
1 pompholyx 29.9 COL17A1 DST
2 herpes gestationis 12.0
3 linear hamartoma syndrome 10.2 COL17A1 DST
4 lipoblastoma 10.2 COL17A1 DST
5 dyskinesia of esophagus 10.2 COL17A1 DST
6 nemaline myopathy 9 10.2 COL17A1 DST
7 epidermolytic acanthoma 10.2 COL17A1 DST
8 atypical autism 10.2 COL17A1 DST
9 bilirubin metabolic disorder 10.2 COL17A1 DST
10 myelitis 10.2 COL17A1 DST
11 nephrosclerosis 10.1 COL17A1 DST
12 oral cavity cancer 10.1 COL17A1 DST
13 nephrotic syndrome, type 2 10.1 COL17A1 DST
14 bullous pemphigoid 10.1
15 peripheral nervous system neoplasm 10.1 COL17A1 DST
16 herpes zoster 10.1 COL17A1 DST
17 ureteral lymphoma 10.1 COL17A1 DST
18 lin-gettig syndrome 10.1 COL17A1 HLA-DRB1
19 intraocular retinoblastoma 10.1 COL17A1 DST
20 mast-cell leukemia 10.1 COL17A1 DST
21 peripheral t-cell lymphoma 10.1 DST HLA-DRB1
22 gilbert syndrome 10.1 COL17A1 DST
23 pol iii-related leukodystrophies 10.1 DST HLA-DRB1
24 natural killer cell leukemia 10.0 CCL11 IL5
25 thelaziasis 10.0 CCL11 IL5
26 lymphoblastic leukemia 10.0 CCL11 IL5
27 spastic diplegia 10.0 CCL11 IL5
28 latent syphilis 10.0 CCL11 IL5
29 necrotizing gastritis 10.0 CCL11 IL5
30 disordered steroidogenesis due to cytochrome p450 oxidoreductase 10.0 CCL11 IL5
31 cerebral primitive neuroectodermal tumor 10.0 CCL11 IL5
32 apricot allergy 10.0 CCL11 IL5
33 trachea leiomyoma 10.0 CCL11 IL5
34 visceral steatosis 10.0 CCL11 IL5
35 cutaneous fibrous histiocytoma 10.0 CCL11 IL5
36 lymphocytic gastritis 10.0 CCL11 IL5
37 intravascular angioleiomyoma 10.0 CCL11 IL5
38 ethmoid sinusitis 10.0 CCL11 IL5
39 childhood malignant mesenchymoma 10.0 CCL11 IL5
40 inflamed seborrheic keratosis 10.0 CCL11 IL5
41 hydrophthalmos 10.0 CCL11 IL5
42 eyelid neoplasm 10.0 CCL11 IL5
43 patellar tendinitis 10.0 CCL11 IL5
44 acquired polycythemia 10.0 CCL11 IL5
45 tracheitis 10.0 CCL11 IL5
46 albinism 10.0 HLA-DRB1 IL5
47 thyroid hyalinizing trabecular adenoma 10.0 CCL11 IL5
48 xanthogranulomatous cholecystitis 10.0 CCL11 IL5
49 colon adenocarcinoma 10.0 CCL11 IL5
50 conjunctival concretion 10.0 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:

56 32 (show all 6)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 pruritus 56 32 Very frequent (99-80%) HP:0000989
2 failure to thrive 56 32 Frequent (79-30%) HP:0001508
3 abnormal blistering of the skin 56 32 Very frequent (99-80%) HP:0008066
4 intrauterine growth retardation 56 32 Frequent (79-30%) HP:0001511
5 premature birth 56 32 Very frequent (99-80%) HP:0001622
6 skin vesicle 56 32 Very frequent (99-80%) HP:0200037

UMLS symptoms related to Pemphigoid Gestationis:


morning sickness

Drugs & Therapeutics for Pemphigoid Gestationis

Interventional clinical trials:


id Name Status NCT ID Phase
1 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Recruiting NCT02874079

Search NIH Clinical Center for Pemphigoid Gestationis

Genetic Tests for Pemphigoid Gestationis

Anatomical Context for Pemphigoid Gestationis

MalaCards organs/tissues related to Pemphigoid Gestationis:

39
Skin, Placenta, T Cells, Testes

Publications for Pemphigoid Gestationis

Articles related to Pemphigoid Gestationis:

(show top 50) (show all 117)
id Title Authors Year
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

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:

id 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:

id Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 8.8 CCL11 HLA-DRB1 IL5

Sources for Pemphigoid Gestationis

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....