Pemphigoid Gestationis disease: Malacards - Research Articles, Drugs, Genes, Clinical Trials

GARD: ¹⁹ 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. 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. 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.

MalaCards based summary: Pemphigoid Gestationis, also known as herpes gestationis, is related to pemphigus gestationis and autoimmune disease, 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 COPI-independent Golgi-to-ER retrograde traffic and Keratinization. Affiliated tissues include skin, placenta and t cells, and related phenotypes are premature birth and pruritus

Disease Ontology: ¹¹ 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.

Orphanet: ⁵⁸ A rare autoimmune bullous skin disease characterized by pruritus with or without polymorphic skin eruption, affecting pregnant women typically during the second and third trimester.

Wikipedia: ⁷⁵ Gestational pemphigoid (GP) is a rare autoimmune variant of the skin disease bullous pemphigoid, and... more...

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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 141)

#	Related Disease	Score	Top Affiliating Genes
1	pemphigus gestationis	30.7	DST DSG3 COL17A1
2	autoimmune disease	30.5	HLA-DRB1 DSG3 DSG1 COL17A1
3	pompholyx	30.4	DST COL17A1
4	epidermolysis bullosa acquisita	30.2	HLA-DRB1 DST COL17A1
5	cicatricial pemphigoid	30.1	DST DSG3 DSG1 COL17A1
6	pemphigoid	30.1	RNASE3 PPL EVPL DST DSG3 DSG1
7	lichen planus	29.9	DST DSG3 DSG1 COL17A1
8	skin disease	29.9	RNASE3 HLA-DRB1 DST DSG3 DSG1 COL17A1
9	celiac disease 1	29.8	TNFRSF25 TNFRSF10A HLA-DRB1
10	bullous pemphigoid	29.8	RNASE3 PPL HLA-DRB1 EVPL DST DSG3
11	subcorneal pustular dermatosis	29.1	PPL EVPL DST DSG3 DSG1
12	pemphigus	28.8	PPL HLA-DRB1 EVPL DST DSG3 DSG1
13	pemphigus foliaceus	28.7	PPL HLA-DRB1 EVPL DST DSG3 DSG1
14	pemphigus vulgaris, familial	28.6	PPL HLA-DRB1 EVPL DST DSG3 DSG1
15	paraneoplastic pemphigus	28.6	PPL EVPL DST DSG3 DSG1 COL17A1
16	bullous skin disease	28.6	PPL EVPL DST DSG3 DSG1 COL17A1
17	pruritic urticarial papules and plaques of pregnancy	10.5
18	dermatitis herpetiformis	10.5
19	exanthem	10.4
20	gestational trophoblastic neoplasm	10.3
21	neurotic excoriation	10.2	DST COL17A1
22	epidermolysis bullosa, junctional 4, intermediate	10.2	DST COL17A1
23	scabies	10.2	DST COL17A1
24	mite infestation	10.2	DST COL17A1
25	junctional epidermolysis bullosa non-herlitz type	10.2	DST COL17A1
26	epidermolysis bullosa, junctional 5b, with pyloric atresia	10.2	DST COL17A1
27	recessive dystrophic epidermolysis bullosa	10.2	DST COL17A1
28	leukocyte adhesion deficiency, type i	10.2
29	hydatidiform mole, recurrent, 1	10.2
30	graves disease 1	10.2
31	erythema multiforme	10.2
32	intrahepatic cholestasis of pregnancy	10.2
33	pre-eclampsia	10.2
34	gestational diabetes	10.2
35	graves' disease	10.2
36	cholestasis	10.2
37	urticaria	10.2
38	intrahepatic cholestasis	10.2
39	dermatitis	10.2
40	choriocarcinoma	10.2
41	lichen planus pemphigoides	10.2
42	kindler syndrome	10.2	DST COL17A1
43	epidermolysis bullosa dystrophica	10.1	DST COL17A1
44	palindromic rheumatism	10.1	TNFRSF10A HLA-DRB1
45	hirata disease	10.1	TNFRSF10A HLA-DRB1
46	acrodermatitis	10.1
47	alopecia	10.1
48	alopecia totalis	10.1
49	congenital herpes simplex	10.1
50	focal epithelial hyperplasia	10.1	TNFRSF10A HLA-DRB1

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

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Symptoms & Phenotypes for Pemphigoid Gestationis

Human phenotypes related to Pemphigoid Gestationis:

⁵⁸ ³⁰ (show all 6)

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

Symptoms:

¹¹

• pruritis

UMLS symptoms related to Pemphigoid Gestationis:

morning sickness

MGI Mouse Phenotypes related to Pemphigoid Gestationis:

⁴⁵

#	Description	MGI Source Accession	Score	Top Affiliating Genes
1	integument	MP:0010771	9.1	COL17A1 DSG1 DSG3 DST EVPL PPL

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Drugs & Therapeutics for Pemphigoid Gestationis

Search Clinical Trials, NIH Clinical Center for Pemphigoid Gestationis

Cochrane evidence based reviews: pemphigoid gestationis

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Genetic Tests for Pemphigoid Gestationis

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Anatomical Context for Pemphigoid Gestationis

Organs/tissues related to Pemphigoid Gestationis:

MalaCards : Skin, Placenta, T Cells, Ovary, Thyroid

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Publications for Pemphigoid Gestationis

Articles related to Pemphigoid Gestationis:

(show top 50) (show all 651)

#	Title	Authors	PMID	Year
1	Pemphigoid gestationis: clinical and laboratory evaluation. ⁵³ ⁶²	Cobo MF...Aoki V	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. ⁵³ ⁶²	Aoyama Y...Kitajima Y	17875879	2007
3	Molecular mapping of the major epitopes of BP180 recognized by herpes gestationis autoantibodies. ⁵³ ⁶²	Lin MS...Giudice GJ	10479533	1999
4	Acquired skin disease of hemidesmosomes. ⁵³ ⁶²	Zillikens D	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. ⁵³ ⁶²	Nakatani C...Shirai T	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. ⁵³ ⁶²	Matsumura K...Hashimoto T	8874743	1996
7	Cicatricial pemphigoid autoantibodies react with multiple sites on the BP180 extracellular domain. ⁵³ ⁶²	Balding SD...Giudice GJ	8592065	1996
8	Molecular mapping of a pathogenically relevant BP180 epitope associated with experimentally induced murine bullous pemphigoid. ⁵³ ⁶²	Liu Z...Giudice GJ	7594563	1995
9	Serum eosinophil cationic protein (ECP) in bullous pemphigoid. ⁵³ ⁶²	Caproni M...Fabbri P	7751092	1995
10	Development of an ELISA to detect anti-BP180 autoantibodies in bullous pemphigoid and herpes gestationis. ⁵³ ⁶²	Giudice GJ...Diaz LA	7516396	1994
11	[Herpes gestationis: immunologic and immunogenetic aspects]. ⁵³ ⁶²	Messer G...Meurer M	8113038	1993
12	A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. ⁵³ ⁶²	Liu Z...Giudice GJ	7693763	1993
13	Bullous pemphigoid and herpes gestationis autoantibodies recognize a common non-collagenous site on the BP180 ectodomain. ⁵³ ⁶²	Giudice GJ...Diaz LA	8228259	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. ⁵³ ⁶²	Li K...Uitto J	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. ⁵³ ⁶²	Diaz LA...Giudice GJ	1698819	1990
16	Histopathological features of pemphigoid gestationis and polymorphic eruption of pregnancy: A blinded retrospective comparative study of 31 cases. ⁶²	Baban F...Johnson EF	36357351	2022
17	Recognizing, Diagnosing, and Managing Pregnancy Dermatoses. ⁶²	Himeles JR...Pomeranz MK	36075066	2022
18	The association of six autoimmune bullous diseases with thyroid disorders: a population-based study. ⁶²	Kridin K...Schmidt E	35611551	2022
19	Pemphigoid gestationis and preeclampsia in a donor-egg IVF pregnancy. ⁶²	Cowan TL...Murrell DF	35919284	2022
20	Acral blisters in neonates: Three rare and benign diseases. ⁶²	Rekik M...Turki H	36245441	2022
21	Successful treatment of recalcitrant pemphigoid gestationis with omalizumab: report of two cases. ⁶²	Konstantinou MP...Bost C	35592910	2022
22	Pseudo-Pemphigoid Gestationis Eruption Following SARS-CoV-2 Vaccination with mRNA Vaccine. ⁶²	de Lorenzi C...Toutous Trellu L	35892478	2022
23	Pemphigoid Gestationis and adverse pregnancy outcomes: A literature review. ⁶²	Abdelhafez MMA...Helmy E	35385801	2022
24	Newborn twins with neonatal pemphigoid gestationis. ⁶²	Kitayama S...Shimizu T	34939222	2022
25	Gestational Pemphigoid Presenting in the Second Trimester of Pregnancy: A Rare Finding. ⁶²	Ghafoor R...Husseni AM	35800842	2022
26	Pruritus in Pregnancy. ⁶²	Stefaniak AA...Stander S	35191007	2022
27	[Skin and pregnancy]. ⁶²	Alvarez Martinez D...Kaya G	35353455	2022
28	Autoreactivity to BP180 Neoepitopes in Patients With Pemphigoid Gestationis. ⁶²	Schauer F...Kiritsi D	34985500	2022
29	Pemphigoid gestationis and polymorphic eruption of pregnancy in skin of color. ⁶²	Xie F...Lehman JS	35064677	2022
30	Annular bullous diseases. ⁶²	Sussman M...Hsu S	34979265	2022
31	Type XVII collagen: Relevance of distinct epitopes, complement-independent effects, and association with neurological disorders in pemphigoid disorders. ⁶²	Opelka B...Goletz S	36032160	2022
32	Influence of pemphigoid gestationis on pregnancy outcome: A case report and review of the literature. ⁶²	Parfene CG...Pelinescu-Onciul D	34815775	2022
33	Diagnosis, fetal risk and treatment of pemphigoid gestationis in pregnancy: A case report. ⁶²	Jiao HN...Zhong HP	35004996	2021
34	Treatment of pemphigoid gestationis with dupilumab. ⁶²	Riquelme-Mc Loughlin C...Mascaro JM	34048080	2021
35	Assessment and management of itchy skin in pregnancy. ⁶²	Ting S...Nixon R	34845477	2021
36	A multicentre prospective analysis of the incidence of pemphigoid diseases in Turkey. ⁶²	Durdu M...Uzun S	34642934	2021
37	Neonatal pemphigoid gestationis: An atypical presentation of a rare disease. ⁶²	Jimenez A...Cipriano SD	34713489	2021
38	The burden of neurological comorbidities in six autoimmune bullous diseases: a population-based study. ⁶²	Kridin K...Schmidt E	34153122	2021
39	Autoimmune bullous diseases in pregnancy: clinical and epidemiological characteristics and therapeutic approach. ⁶²	Fagundes PPS...Aoki V	34304937	2021
40	Subepithelial autoimmune blistering dermatoses: Clinical features and diagnosis. ⁶²	Montagnon CM...Lehman JS	33684496	2021
41	Severe pemphigoid gestationis responsive to intravenous immunoglobulins after initial treatment failure of combined oral steroids and cyclosporin. ⁶²	Killion L...O'Brennan E	34001471	2021
42	Pemphigoid gestationis: a rare pregnancy dermatosis treated with a combination of IVIg and rituximab. ⁶²	Narayanan A...Bhari N	33727302	2021
43	Pemphigoid Gestationis - Case Report and Review of Literature. ⁶²	Ceryn J...Lesiak A	34168478	2021
44	Intravenous Immunoglobulin Therapy for Gestational Pemphigoid Refractory to Conventional Treatment. ⁶²	Boria F...de la Calle M	32986975	2021
45	Flame Figures in Linear Immunoglobulin A Bullous Dermatosis Secondary to Parvovirus B19 Infection. ⁶²	Garcia-Gil MF...Prieto-Torres L	32472771	2021
46	The epidemiology of autoimmune bullous diseases in Sudan between 2000 and 2016. ⁶²	Siddig O...Suleiman AM	34255799	2021
47	Pemphigoid Gestationis-A Rare Pregnancy Dermatoses. ⁶²	Dixit N...Singh P	33417630	2020
48	A Rare Case of Recurrent Postpartum Pemphigoid Gestationis with Fetal Complications and Skip Pregnancy. ⁶²	Puri N...Singh O	33344355	2020
49	Pemphigoid Gestationis. ⁶²	Agostinis P...Antonello RM	32846065	2020
50	A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita. ⁶²	Patel PM...Amber KT	32180161	2020

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Genes for Pemphigoid Gestationis

Genes related to Pemphigoid Gestationis (0 elite genes):

(show all 11)

- Elite gene

- Cancer Census gene in COSMIC

#	Symbol	Description	Category	Score	Evidence	PubMed IDs
1	COL17A1	Collagen Type XVII Alpha 1 Chain	Protein Coding	29.09	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	8228259 8592065 10479533 (more) 8113038 19936176 1512460 8874743 10379705 7594563 7693763 1698819 9767278 7516396
2	DST	Dystonin	Protein Coding	26.36	DISEASES inferred ¹⁴ Novoseek inferred ⁵³ GeneCards inferred via : Publications (show sections)	8874743 17875879 1512460
3	HLA-DRB1	Major Histocompatibility Complex, Class II, DR Beta 1	Protein Coding	11.26	DISEASES inferred ¹⁴ GeneCards inferred via : Publications (show sections)
4	RNASE3	Ribonuclease A Family Member 3	Protein Coding	10.42	DISEASES inferred ¹⁴ Novoseek inferred ⁵³	7751092
5	TNFRSF25	TNF Receptor Superfamily Member 25	Protein Coding	7.02	DISEASES inferred ¹⁴
6	DSG3	Desmoglein 3	Protein Coding	6.77	DISEASES inferred ¹⁴
7	TNFRSF10A	TNF Receptor Superfamily Member 10a	Protein Coding	6.73	DISEASES inferred ¹⁴
8	DSG1	Desmoglein 1	Protein Coding	6.71	DISEASES inferred ¹⁴
9	EVPL	Envoplakin	Protein Coding	5.53	DISEASES inferred ¹⁴
10	PPL	Periplakin	Protein Coding	5.53	DISEASES inferred ¹⁴
11	RNF103	Ring Finger Protein 103	Protein Coding	5.49	DISEASES inferred ¹⁴

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Variations for Pemphigoid Gestationis

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Expression for Pemphigoid Gestationis

Search GEO for disease gene expression data for Pemphigoid Gestationis.

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Pathways for Pemphigoid Gestationis

Pathways related to Pemphigoid Gestationis according to GeneCards Suite gene sharing:

#	Super pathways	Score	Top Affiliating Genes
1	COPI-independent Golgi-to-ER retrograde traffic ⁶⁶ Show member pathways Cell cycle Spindle assembly and chromosome separation ²² Cytoskeleton remodeling Keratin filaments ²² Cytoskeleton remodeling Neurofilaments ²² HSP90 chaperone cycle for steroid hormone receptors (SHR) in the presence of ligand ⁶⁶	11.62	PPL EVPL DST
2	Keratinization ⁶⁶ Show member pathways Formation of the cornified envelope ⁶⁶	11.46	PPL EVPL DSG3 DSG1
3	Validated transcriptional targets of TAp63 isoforms ⁶¹	10.89	EVPL DST

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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	membrane	GO:0016020	10.36	TNFRSF25 TNFRSF10A RNF103 HLA-DRB1 DST DSG3
2	membrane	GO:0016021	10.36	TNFRSF25 TNFRSF10A RNF103 HLA-DRB1 DST DSG3
3	intermediate filament	GO:0005882	9.92	DST EVPL HLA-DRB1 PPL
4	cornified envelope	GO:0001533	9.76	PPL EVPL DSG3 DSG1
5	hemidesmosome	GO:0030056	9.67	DST COL17A1
6	cellular anatomical entity	GO:0110165	9.65	TNFRSF25 TNFRSF10A PPL EVPL DST
7	anchoring junction	GO:0070161	9.43	PPL EVPL DST DSG3 DSG1 COL17A1
8	desmosome	GO:0030057	9.23	PPL EVPL DSG3 DSG1

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

#	Name	GO ID	Score	Top Affiliating Genes
1	epidermis development	GO:0008544	9.73	HLA-DRB1 EVPL COL17A1
2	wound healing	GO:0042060	9.63	PPL EVPL DST
3	hemidesmosome assembly	GO:0031581	9.26	DST COL17A1
4	intermediate filament cytoskeleton organization	GO:0045104	9.1	PPL EVPL DST

Molecular functions related to Pemphigoid Gestationis according to GeneCards Suite gene sharing:

#	Name	GO ID	Score	Top Affiliating Genes
1	structural molecule activity	GO:0005198	9.1	PPL EVPL DST

Sources

Sources for Pemphigoid Gestationis

¹ BitterDB

² CDC

³ Cell Signaling Technology

⁴ ClinicalTrials

⁵ ClinVar

⁶ CNVD

⁷ Cochrane Library

⁸ Cosmic

⁹ dbSNP

¹⁰ DGIdb

¹¹ Disease Ontology

¹² diseasecard

¹³ DiseaseEnhancer

¹⁴ DISEASES

¹⁵ DrugBank

¹⁶ EFO

¹⁷ ExPASy

¹⁸ FMA

¹⁹ GARD

²⁰ GeneAnalytics

²¹ GeneCards

²² GeneGo (Thomson Reuters)

²³ GeneHancer via GeneCards

²⁴ GeneReviews

²⁵ GenomeRNAi

²⁶ GEO DataSets

²⁷ GO

²⁸ GTR

²⁹ HMDB

³⁰ HPO

³¹ ICD10

³² ICD10 via Orphanet

³³ ICD11

³⁴ ICD9CM

³⁵ IUPHAR

³⁶ LifeMap

³⁷ LncRNADisease

³⁸ LOVD

³⁹ MalaCards

⁴⁰ MedGen

⁴¹ MedlinePlus

⁴² MedlinePlus Genetics

⁴³ MeSH

⁴⁴ MESH via Orphanet

⁴⁵ MGI

⁴⁶ miR2Disease

⁴⁷ NCBI Bookshelf

⁴⁸ NCI

⁴⁹ NCIt

⁵⁰ NDF-RT

⁵¹ NIH Clinical Center

⁵² NINDS

⁵³ Novoseek

⁵⁴ Novus Biologicals

⁵⁵ ODiseA

⁵⁶ OMIM via Orphanet

⁵⁷ OMIM® (Updated 08-Dec-2022)

⁵⁸ Orphanet

⁵⁹ PathCards

⁶⁰ PharmGKB

⁶¹ PubChem

⁶² PubMed

⁶³ PubMed Health

⁶⁴ QIAGEN

⁶⁵ R&D Systems

⁶⁶ Reactome

⁶⁷ Sino Biological

⁶⁸ SNOMED-CT

⁶⁹ SNOMED-CT via HPO

⁷⁰ Tocris

⁷¹ UMLS

⁷² UMLS via Orphanet

⁷³ UniProtKB/Swiss-Prot

⁷⁴ WikiPathways

⁷⁵ Wikipedia

MCID: PMP002 MIFTS: 40	Pemphigoid Gestationis Categories: Immune diseases, Rare diseases, Skin diseases	Data Licensing For inquiries, contact: [email protected]
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