MCID: PMP001
MIFTS: 55

Pemphigus

Categories: Cancer diseases, Genetic diseases, Immune diseases, Oral diseases, Rare diseases, Skin diseases

Aliases & Classifications for Pemphigus

MalaCards integrated aliases for Pemphigus:

Name: Pemphigus 12 74 52 36 54 42 15 17 71 32

Classifications:



External Ids:

Disease Ontology 12 DOID:9182
KEGG 36 H01648
ICD9CM 34 694.4
MeSH 43 D010392
NCIt 49 C34909
SNOMED-CT 67 65172003
ICD10 32 L10 L10.9
UMLS 71 C0030807

Summaries for Pemphigus

KEGG : 36 Pemphigus is an autoimmune blistering disease of the skin and mucous membranes characterized by circulating autoantibodies that target desmosomal components as well as other epithelial antigens and impair keratinocytes adhesion with subsequent acantholysis. Although the precise pathomechanisms of pemphigus have not yet been fully elucidated, recent studies have shown that desmosome disassembling involves not only the direct inhibition of desmosome formation caused by IgGs, but also desmosome remodeling. Pemphigus can be divided into non-inflammatory types, which are the major and common types, and inflammatory types, which are much less common and include necrolytic (apoptotic) type. Non-inflammatory pemphigus includes two types: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). The former is caused by autoantibodies to desmoglein (Dsg) 3 only or to both Dsg1 and Dsg3 and generates clinically multiple flaccid blisters and erosions due to acantholysis at the lower, typically along the suprabasal, layers in the epidermis and mucous membranes. The latter is caused by autoantibodies to Dsg1 and clinically characterized by very fragile superficial blisters and large leafy scales due to acantholysis at the granular cell layer in the epidermis, but not observed in the mucous membranes. Inflammatory types of pemphigus include pemphigus herpetiformis (PH), pemphigus vegetans (PVeg) and the so-called paraneoplastic pemphigus (PP). Because pemphigus is an autoimmune disease, the most common treatment is the administration of oral steroids to inhibit antibody production, combined with the use of topical agents to prevent infection, protect the eroded areas and promote epithelialization. Other concomitant therapy can include the use of immunosuppressants, plasma exchange, and the administration of high-dose intravenous immunoglobulin (IVIG).

MalaCards based summary : Pemphigus is related to pemphigus erythematosus and pemphigus vegetans, and has symptoms including pruritus and exanthema. An important gene associated with Pemphigus is DSG3 (Desmoglein 3), and among its related pathways/superpathways are Developmental Biology and Arrhythmogenic right ventricular cardiomyopathy (ARVC). The drugs Dexamethasone and Dexamethasone acetate have been mentioned in the context of this disorder. Affiliated tissues include skin, testes and t cells, and related phenotypes are Decreased viability and Decreased viability

NIH Rare Diseases : 52 Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes (mouth, nose, throat, eyes, and genitals). This condition can occur at any age, but often strikes people in middle or older age. Studies have shown that some populations may be at greater risk for certain types of pemphigus. For instance, people of Jewish descent and those from India, Southeast Europe, and the Middle East are at greater risk for pemphigus vulargis, while pemphigus foliaceus is more common in North America, Turkey, and South America. Pemphigus is a chronic disease which is best controlled by early diagnosis and treatment. Treatment includes steroids to reduce inflammation, drugs that suppress the immune system response and antibiotics to treat associated infections. There are four main types of pemphigus: Pemphigus vulgaris Pemphigus foliaceus IgA pemphigus Paraneoplastic pemphigus

MedlinePlus : 42 Pemphigus is an autoimmune disorder. If you have it, your immune system attacks healthy cells in your skin and mouth, causing blisters and sores. No one knows the cause. Pemphigus does not spread from person to person. It does not appear to be inherited. But some people's genes put them more at risk for pemphigus. Pemphigoid is also an autoimmune skin disease. It leads to deep blisters that do not break easily. Pemphigoid is most common in older adults and may be fatal for older, sick patients. Doctors diagnose pemphigus with a physical exam, a biopsy, and blood tests. The treatment of pemphigus and pemphigoid is the same: one or more medicines to control symptoms. These may include Steroids, which reduce inflammation Drugs that suppress the immune system response Antibiotics to treat associated infections NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Wikipedia : 74 Pemphigus (/'p?mf?g?s/ or /p?m'fa?g?s/) is a rare group of blistering autoimmune diseases that affect... more...

Related Diseases for Pemphigus

Diseases in the Pemphigus family:

Benign Chronic Pemphigus

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

(show top 50) (show all 661)
# Related Disease Score Top Affiliating Genes
1 pemphigus erythematosus 34.9 DSP DSG3
2 pemphigus vegetans 34.8 PPL DSG1 DSC3
3 herpetiform pemphigus 34.2 DST DSG3 DSG1
4 pemphigus foliaceus 33.9 PPL HLA-DRB1 EVPL DST DSG3 DSG2
5 iga pemphigus 33.7 PPL EVPL DSG3 DSG1 DSC3 DSC1
6 pemphigus gestationis 33.5 DST DSP DSG3 DSG1 COL17A1
7 benign chronic pemphigus 32.6 JUP DSP DSG4 DSG3 DSG2 DSG1
8 epidermolysis bullosa, lethal acantholytic 32.4 JUP DSP
9 pemphigoid gestationis 32.3 HLA-DRB1 DST DSG3 DSG1 COL17A1
10 cicatricial pemphigoid 32.2 HLA-DRB1 DST COL17A1 CD79A
11 bullous pemphigoid 32.2 PPL HLA-DRB1 EVPL DST DSP DSG3
12 paraneoplastic pemphigus 32.0 PPL HLA-DRB1 EVPL DST DSP DSG3
13 ritter's disease 31.9 DSG4 DSG3 DSG2 DSG1 DSC3 DSC2
14 subcorneal pustular dermatosis 31.9 PPL EVPL DST DSP DSG3 DSG1
15 pemphigus vulgaris, familial 31.5 PPL PLAUR JUP HLA-DRB1 EVPL DST
16 autoimmune disease 31.4 HLA-DRB1 DSG3 DSG1 COL17A1 CD79A
17 linear iga disease 31.4 DST COL17A1 CD79A
18 bullous impetigo 31.0 DSG3 DSG1
19 follicular dendritic cell sarcoma 30.9 PPL EVPL DSP DSG3
20 lichen planus 30.9 DST DSG3 DSG1 COL17A1
21 castleman disease 30.9 PPL EVPL DSP DSG3 DSG1
22 grover's disease 30.8 JUP DSP
23 ocular cicatricial pemphigoid 30.7 HLA-DRB1 CD79A
24 keratosis 30.5 DST DSP DSG4 DSG2 DSG1
25 lichen planus pemphigoides 30.5 DST COL17A1
26 epidermolysis bullosa 30.4 DST DSP COL17A1
27 impetigo 30.4 DSG4 DSG3 DSG2 DSG1 DSC3 DSC1
28 hidradenitis 30.1 DSG3 DSG1 DSC2 DSC1
29 pseudomembranous conjunctivitis 30.1 EVPL DSP A2ML1
30 hidradenitis suppurativa 30.0 DSG3 DSG1 DSC2 DSC1
31 epidermolysis bullosa acquisita 29.9 HLA-DRB1 DST COL17A1
32 skin disease 29.8 DST DSP DSG4 DSG3 DSG2 DSG1
33 hypotrichosis 29.4 DSG4 DSG1 DSC3 CDH3
34 left bundle branch hemiblock 29.4 JUP DSP DSG2 DSC2
35 brugada syndrome 29.1 JUP DSP DSG2 DSC2
36 darier-white disease 29.0 JUP DSP DSG4 DSG3 DSC3 DSC2
37 bullous skin disease 28.5 PPL EVPL DST DSP DSG4 DSG3
38 cellulitis 28.1 PPL EVPL DST DSP DSG4 DSG3
39 pemphigus and fogo selvagem 12.5
40 superficial pemphigus 12.4
41 recessive dystrophic epidermolysis bullosa-generalized other 11.2
42 dermatitis 10.7
43 dermatitis herpetiformis 10.7
44 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.7
45 dermatitis herpetiformis, familial 10.6
46 thymoma, familial 10.6
47 thymoma 10.6
48 myasthenia gravis 10.5
49 pustulosis of palm and sole 10.5
50 psoriasis 10.5

Comorbidity relations with Pemphigus via Phenotypic Disease Network (PDN):


Acute Cystitis Bullous Pemphigoid
Decubitus Ulcer Heart Disease

Graphical network of the top 20 diseases related to Pemphigus:



Diseases related to Pemphigus

Symptoms & Phenotypes for Pemphigus

UMLS symptoms related to Pemphigus:


pruritus, exanthema

GenomeRNAi Phenotypes related to Pemphigus according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased viability GR00221-A-1 10.05 PLAUR
2 Decreased viability GR00221-A-2 10.05 PLAUR
3 Decreased viability GR00221-A-4 10.05 PLAUR
4 Decreased viability GR00381-A-1 10.05 CD79A
5 Decreased viability GR00381-A-3 10.05 CD79A
6 Decreased viability GR00402-S-2 10.05 A2ML1 ANXA9 ATP2C1 CD79A CDH3 COL17A1
7 no effect GR00402-S-1 9.62 A2ML1 ANXA9 ATP2C1 CD79A CDH3 COL17A1

MGI Mouse Phenotypes related to Pemphigus:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.4 ATP2C1 CDH3 DSC1 DSC3 DSG1 DSG2

Drugs & Therapeutics for Pemphigus

Drugs for Pemphigus (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 116)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Dexamethasone Approved, Investigational, Vet_approved Phase 2, Phase 3 50-02-2 5743
2
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2, Phase 3 1177-87-3
3
Triamcinolone Approved, Vet_approved Phase 3 124-94-7 31307
4
Mycophenolic acid Approved Phase 3 24280-93-1 446541
5
rituximab Approved Phase 3 174722-31-7 10201696
6
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
7
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
8
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
9
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
10 Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
11
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
12
ofatumumab Approved Phase 3 679818-59-8 6918251
13
Acetaminophen Approved Phase 3 103-90-2 1983
14
Cetirizine Approved Phase 3 83881-51-0 2678
15
Histamine Approved, Investigational Phase 3 51-45-6 774
16
Tyrosine Approved, Investigational, Nutraceutical Phase 3 60-18-4 6057
17
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
18 HIV Protease Inhibitors Phase 2, Phase 3
19
protease inhibitors Phase 2, Phase 3
20 BB 1101 Phase 2, Phase 3
21 triamcinolone acetonide Phase 3
22 Triamcinolone diacetate Phase 3
23 Triamcinolone hexacetonide Phase 3
24 Antitubercular Agents Phase 3
25 Rho(D) Immune Globulin Phase 3
26 Immunoglobulins, Intravenous Phase 3
27 gamma-Globulins Phase 3
28 Antineoplastic Agents, Immunological Phase 3
29 Antirheumatic Agents Phase 3
30 Immunologic Factors Phase 3
31 Immunoglobulins Phase 3
32 Antibodies Phase 3
33 Antiemetics Phase 3
34 Gastrointestinal Agents Phase 3
35 Hormone Antagonists Phase 3
36 Autonomic Agents Phase 3
37 Anti-Inflammatory Agents Phase 3
38 Antineoplastic Agents, Hormonal Phase 3
39 glucocorticoids Phase 3
40 Hormones Phase 3
41 Analgesics, Non-Narcotic Phase 3
42 Analgesics Phase 3
43 Methylprednisolone Acetate Phase 3
44 Antibodies, Monoclonal Phase 3
45 Neurotransmitter Agents Phase 3
46 Antipyretics Phase 3
47
Histamine Phosphate Phase 3 51-74-1 65513
48 Neuroprotective Agents Phase 3
49 Histamine Antagonists Phase 3
50 Histamine H1 Antagonists Phase 3

Interventional clinical trials:

(show top 50) (show all 58)
# Name Status NCT ID Phase Drugs
1 A PILOT STUDY TO ASSESS THE EFFICACY OF RITUXIMAB VERSUS COMBINATION OF RITUXIMAB AND INTRAVENOUS CYCLOPHOSPHAMIDE IN THE TREATMENT OF REFRACTORY PEMPHIGUS Unknown status NCT01974518 Phase 3 Rituximab and Cyclophosphamide IV
2 EVALUATION CLINIQUE ET BIOLOGIQUE A LONG TERME DES PATIENTS ATTEINTS DE FORMES SEVERES DE PEMPHIGUS TRAITES PAR LE RITUXIMAB Completed NCT01299857 Phase 3 Rituximab
3 Traitment of Patients Presenting Pemphigus With Anti CD20 (Mabthera). Completed NCT00213512 Phase 2, Phase 3 Mabthera
4 Comparative Study Between Intralesional Autologous Platelet Rich Plasma and Intralesional Triamcinolone Acetonide in the Oral Erosions of Pemphigus Vulgaris Patients Completed NCT02828163 Phase 3 Triamcinolone Acetonide
5 Pharmacokinetics Study of Mycophenolic Acid in Patients With an Autoimmune Bullous Dermatose, Pemphigus or Cicatricial Pemphigoid. Completed NCT02993133 Phase 3 Cellcept® in autoimmune bullous dermatoses
6 European Randomised Placebo-Controlled Trial of Adjuvant Oral Glucocorticoid Pulse Therapy in Pemphigus (Pempuls Trial) Completed NCT00127764 Phase 2, Phase 3 dexamethasone (50mg 1dd6, 3 consecutive days/month)
7 Comparison Between Monoclonal Antibody CD20 Treatment (Rituximab (mabthéra))and General Corticotherapy Treatment in Patients With Pemphigus Completed NCT00784589 Phase 3 General Corticotherapy;Rituximab
8 A Prospective, Randomised, Double-blind, Placebo-controlled, Parallel Group, Mult-center, 52-week Trial to Assess the Efficacy and Safety of Adjunct Mycophenolate Mofetil (MMF) to Achieve Remission With Reduced Corticosteroid in Subjects With Pemphigus Vulgaris Completed NCT00683930 Phase 3 Mycophenolate Mofetil 2 g/Day;Mycophenolate Mofetil (MMF) 3 g/Day;Placebo
9 A Randomized, Double-Blind, Double-Dummy, Active-Comparator, Multicenter Study to Evaluate the Efficacy and Safety of Rituximab Versus MMF in Patients With Pemphigus Vulgaris Completed NCT02383589 Phase 3 Mycophenolate Mofetil Placebo;Mycophenolate Mofetil;Rituximab;Rituximab Placebo
10 NPB-01(Intravenous Immunoglobulin) Therapy for Patients With Bullous Pemphigoid Unresponsive to Corticosteroids: Randomized, Double-Blind, Placebo Control,Parallel Assignment Study(Phase III) Completed NCT01408550 Phase 3 NPB-01;Placebo
11 A Randomized, Double-Blind, Placebo-Controlled, Multicenter Trial to Evaluate the Efficacy and Safety of Oral BTK Inhibitor PRN1008 in Moderate to Severe Pemphigus Recruiting NCT03762265 Phase 3 PRN1008 Oral Tablet;Placebo Oral Tablet
12 Clinical and Immunological Long-term Follow-up of Patients With Pemphigus Included in the "RITUXIMAB 3" Trial With Medico-economic Evaluation of the Treatment. Not yet recruiting NCT03790293 Phase 3 Rituximab;corticosteroids'therapy
13 OPV116910: A Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Investigate the Efficacy and Safety of Ofatumumab Injection for Subcutaneous Use in Subjects With Pemphigus Vulgaris Terminated NCT01920477 Phase 3
14 OPV117059: A Long-Term Extension Study of Ofatumumab Injection for Subcutaneous Use in Subjects With Pemphigus Vulgaris Terminated NCT02613910 Phase 3 Ofatumumab;Acetaminophen/paracetamol;Antihistamine (cetirizine or equivalent);Prednisone/Prednisolone
15 A Randomized, Partial-blind, Placebo-controlled Trial Evaluating the Efficacy, Safety, Pharmacokinetics and Pharmacodynamics of VAY736 in the Treatment of Patients With Pemphigus Vulgaris Completed NCT01930175 Phase 2 VAY736;Placebo to VAY736;VAY736
16 Phase II Study of High-Dose Cyclophosphamide in Patients With Refractory Pemphigus Completed NCT00010413 Phase 2 cyclophosphamide;filgrastim
17 Combined Treatment of Autoimmune Bullous Diseases With Protein A Immunoadsorption, Dexamethasone Pulse Therapy and Rituximab Completed NCT00656656 Phase 2 Combination of Protein A Immunoadsorption, Rituximab, Dexamethasone plus Azathioprine
18 A Phase 2 Open-Label Uncontrolled Pilot Study of KC706 in Patients With Stable, Active Pemphigus Vulgaris Completed NCT00606749 Phase 2 KC706
19 A Double-Blinded, Placebo-Controlled Study of Etanercept (Enbrel) in the Treatment of Pemphigus Vulgaris Completed NCT00135720 Phase 2 Enbrel (Etanercept)
20 A Randomized, Double-Blind, Placebo-Controlled Phase II Trial of Adjuvant Azathioprine Therapy in New Cases of Pemphigus Vulgaris Receiving Prednisone Completed NCT00626678 Phase 2 Azathioprine;Prednisone;Placebo
21 A Randomized, Double-Blind, Placebo-Controlled Phase II Trial of Infliximab in Subjects With Pemphigus Vulgaris Receiving Prednisone Completed NCT00283712 Phase 2 Infliximab
22 Phase I/II Clinical Trial Evaluating Rituximab in Ocular Cicatricial Pemphigoid Completed NCT00584935 Phase 1, Phase 2 Rituximab
23 Rituximab in the Treatment of Patients With Bullous Pemphigoid Completed NCT00286325 Phase 1, Phase 2 Rituximab
24 An Open-label, Non-controlled, Phase II Study to Evaluate the Safety, Pharmacodynamics, Pharmacokinetics, Efficacy and Conditions of Use of ARGX-113 in Patients With Mild to Moderate Pemphigus (Vulgaris and Foliaceus) Recruiting NCT03334058 Phase 2 ARGX-113
25 An Open-Label, Phase 2, Pilot Study Investigating the Safety, Clinical Activity, Pharmacokinetics, and Pharmacodynamics of Oral Treatment With the BTK Inhibitor PRN1008 in Patients With Newly Diagnosed or Relapsing Pemphigus Vulgaris Active, not recruiting NCT02704429 Phase 2 PRN1008
26 TOPical Sirolimus in linGUal Microcystic Lymphatic Malformation -TOPGUN Active, not recruiting NCT04128722 Phase 2 Sirolimus Oral Liquid Product 1mg/mL
27 Short-term Efficacy of Interleukin-2 Gargle Combined With Systemic Use of Glucocorticoids in the Treatment of Oral Mucosal Lesion in Pemphigus Vulgaris: a Randomized, Controlled, Double-blind, Multicenter Clinical Study Not yet recruiting NCT04023149 Phase 2 recombinant human interleukin-2 (rhIL-2);placebo
28 A Phase 1b/2, Multicenter, Open-Label, Safety, and Dose-Finding Study of SYNT001 in Subjects With Pemphigus (Vulgaris or Foliaceus) Terminated NCT03075904 Phase 1, Phase 2 SYNT001
29 Phase 2 Randomized Trial of IVIg With or Without Cyclophosphamide in Pemphigus Terminated NCT00483119 Phase 2 intravenous immunoglobulin;cyclophosphamide
30 A Prospective Randomized Placebo-Controlled Clinical Trial of Dapsone as a Glucocorticoid-Sparing Agent in Maintenance Phase Pemphigus Vulgaris Terminated NCT00429533 Phase 2 Dapsone
31 A Phase 2 Dose-Escalation Study of the Safety and Tolerability of INCB050465 in Participants With Pemphigus Vulgaris Withdrawn NCT03780166 Phase 2 Parsaclisib
32 An Open-Label, Dose-Escalation, Phase I Clinical Study to Assess the Safety of PI-0824 in Patient With Pemphigus Vulgaris Requiring Daily Corticosteroid Therapy Completed NCT00063752 Phase 1 PI-0824
33 Evaluation of the Improvement of Quality of Life of Patients Suffering From Hailey Hailey or Darier Disease After Injections of Botulism Toxin Into Large Folds. Toxin Hailey Darier Completed NCT02782702 Phase 1 Botulism Toxin Treatment
34 Pharmacokinetics and Distribution of Dapsone (DDS) in Leucocytes After Single-dose and Multiple-dose Administration in Healthy Subjects Genotyped for CYP2C9 and NAT2 and in Patients With Autoimmune Bullous Dermatoses Completed NCT02493283 Phase 1 Dapsone single dose;Dapsone multiple dose
35 A Phase I, Open-Label, Multicenter Trial Exploring the Safety and Tolerability of Autologous Polyclonal Regulatory T Cell Therapy in Adults With Active Pemphigus (APG01) Recruiting NCT03239470 Phase 1
36 Rituximab (Anti-CD20) for the Treatment of Subjects With Anticytokine Autoantibody-Associated Diseases Active, not recruiting NCT01842386 Phase 1 Rituximab
37 High Dose Cyclophosphamide & ATG With Hematopoietic Stem Cell Support in Patients With Autoimmune Bullous Skin Disorders: A Phase I Trial Terminated NCT00278642 Phase 1
38 Treatment of Moderate to Severe Patients With Pemphigus With the Monoclonal Anti CD20 Antibody Rituximab at a Protocol of 1000mgX2 and Assessment of Their Immune Status Via the Cylex Test Unknown status NCT01338103 Rituximab
39 Identification of Vulnerability Factors in the Course of Pemphigus Patients - SHS Pemphigus Clinical Trial Unknown status NCT02237313
40 Serum IL-21 Levels in Patients With Pemphigus Vulgaris Unknown status NCT03177213
41 Genetic Susceptibility and Influence of the Microbiomae in Bullous Pemphigoid Unknown status NCT02874079
42 An Open Randomized Comparative Trial of Four Different Dressings for Cutaneous Coverage in Pemphigus and Pemphigoid Unknown status NCT02365675
43 Serum Free Fatty Acids Desaturase Activity Indices in Sera of Patients on Long-term High-dose Corticosteroid Treatment Unknown status NCT02872584 Prednisone
44 Low Dose (1mcg) ACTH Stimulation Test for Assessment of the Hypothalamo-Pituitary- Adrenal Axis in Patients Treated With Topical Corticosteroids Unknown status NCT00476489
45 Research Registry for Inherited Disorders of Keratinization Unknown status NCT00074685
46 Comparison of Two Therapeutic Strategies of Dermoval in Treatment of Bullous Pemphigus Completed NCT00213421 Dermoval
47 The RITAI Cohort. An Observational Study on Serious Adverse Events Occuring After Rituximab Off-label Use for Auto-immune Disorders Completed NCT00960713 Rituximab (MABTHERA® or RITUXAN®).
48 Register of Autoimmune Bullous Dermatoses Completed NCT02899923
49 A Controlled Cross Sectional Study: Prevalence of Cutaneous Autoimmune Phenomena in HIV Infected Patients Completed NCT03129308
50 Immunological Changes in Chronic Graft Versus Host Disease Treated With Extracorporeal Photopheresis Completed NCT01174277

Search NIH Clinical Center for Pemphigus

Inferred drug relations via UMLS 71 / NDF-RT 50 :


Auranofin
Aurothioglucose
Chloroquine
Chloroquine hydrochloride
chloroquine phosphate
Potassium aminobenzoate

Genetic Tests for Pemphigus

Anatomical Context for Pemphigus

MalaCards organs/tissues related to Pemphigus:

40
Skin, Testes, T Cells, B Cells, Lung, Eye, Thyroid

Publications for Pemphigus

Articles related to Pemphigus:

(show top 50) (show all 8475)
# Title Authors PMID Year
1
Pemphigus. 61 42
31498102 2019
2
Assessment of Diagnostic Strategy for Early Recognition of Bullous and Nonbullous Variants of Pemphigoid. 42
30624575 2019
3
Biochip detection of BP180 autoantibodies in blister fluid for the serodiagnosis of bullous pemphigoid: A pilot study. 42
30762788 2019
4
C3d immunohistochemistry on formalin-fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin. 54 61
19863700 2010
5
Novel ELISA systems for antibodies to desmoglein 1 and 3: correlation of disease activity with serum autoantibody levels in individual pemphigus patients. 54 61
20163452 2010
6
p38MAPK signaling and desmoglein-3 internalization are linked events in pemphigus acantholysis. 54 61
20093368 2010
7
A case of pemphigus herpetiformis with absence of antibodies to desmogleins 1 and 3. 54 61
20050294 2010
8
Autoantibodies against desmocollins in European patients with pemphigus. 54 61
19456767 2009
9
Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus. 54 61
19737550 2009
10
Antibodies to the desmoglein 1 precursor proprotein but not to the mature cell surface protein cloned from individuals without pemphigus. 54 61
19843946 2009
11
Dermatoses affecting desmosomes in animals: a mechanistic review of acantholytic blistering skin diseases. 54 61
20178467 2009
12
Detection of anti-envoplakin and anti-periplakin autoantibodies by ELISA in patients with paraneoplastic pemphigus. 54 61
18820940 2009
13
[IgA pemphigus accompanying multiple myeloma has disappeared following the treatment with bortezomib (Velcade), cyclophosphamide and dexamethasone. Case study and literature review]. 54 61
19947244 2009
14
Desmocollin 3-mediated binding is crucial for keratinocyte cohesion and is impaired in pemphigus. 54 61
19717567 2009
15
Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. 54 61
19841404 2009
16
Oral pemphigus. 54 61
19893475 2009
17
Immunogenicity of rituximab in patients with severe pemphigus. 54 61
19502112 2009
18
Neonatal pemphigus in an infant born to a mother with serologic evidence of both pemphigus vulgaris and gestational pemphigoid. 54 61
19467379 2009
19
The value of desmoglein 1 and 3 antibody ELISA testing in patients with pemphigus. 54 61
19451505 2009
20
An 'n-of-1' placebo-controlled crossover trial of intravenous immunoglobulin as adjuvant therapy in refractory pemphigus vulgaris. 54 61
19222460 2009
21
Disruption of desmosome assembly by monovalent human pemphigus vulgaris monoclonal antibodies. 54 61
19037235 2009
22
Peptides Targeting the Desmoglein 3 Adhesive Interface Prevent Autoantibody-induced Acantholysis in Pemphigus. 54 61
19164289 2009
23
Role of autoantibodies against the linker subdomains of envoplakin and periplakin in the pathogenesis of paraneoplastic pemphigus. 54 61
19323896 2009
24
High-dose pemphigus antibodies against linear epitopes of desmoglein 3 (Dsg3) can induce acantholysis and depletion of Dsg3 from keratinocytes. 54 61
19200441 2009
25
Discohesive malignant melanoma simulating a bullous dermatoses. 54 61
19208079 2009
26
Detection of autoantibodies against recombinant desmoglein 1 and 3 molecules in patients with pemphigus vulgaris: correlation with disease extent at the time of diagnosis and during follow-up. 54 61
20049340 2009
27
Pemphigus: the promises of peptide immunotherapy. 54 61
19874216 2009
28
Detection and characterization of anti-envoplakin linker autoantibodies in paraneoplastic pemphigus using specific bead-based assay. 54 61
18790674 2008
29
Immunostaining with antibodies to desmoglein provides the diagnosis of drug-induced pemphigus and allows prediction of outcome. 54 61
18701409 2008
30
Pemphigus vulgaris IgG directly inhibit desmoglein 3-mediated transinteraction. 54 61
18641320 2008
31
E-cadherin is an additional immunological target for pemphigus autoantibodies. 54 61
18219275 2008
32
Immunoglobulin A pemphigus associated with immunoglobulin A gammopathy and lung cancer. 54 61
18578711 2008
33
Evaluating efficacy of plasmapheresis for patients with pemphigus using desmoglein enzyme-linked immunosorbent assay. 54 61
18241273 2008
34
Enzyme-linked immunosorbent assay in autoimmune blistering diseases: preliminary experience of the Dermatology Department of Cagliari. 54 61
18833046 2008
35
Beyond steric hindrance: the role of adhesion signaling pathways in the pathogenesis of pemphigus. 54 61
17574391 2007
36
A perspective of pemphigus from bedside and laboratory-bench. 54 61
18094947 2007
37
Pemphigus IgG causes skin splitting in the presence of both desmoglein 1 and desmoglein 3. 54 61
17640963 2007
38
Immunoglobulin A pemphigus involving the perianal skin and oral mucosa: an unusual presentation. 54 61
17956010 2007
39
Intraperitoneal injection of pemphigus vulgaris-IgG into mouse depletes epidermal keratinocytes of desmoglein 3 associated with generation of acantholysis. 54 61
17431647 2007
40
IgG autoantibodies directed against desmoglein 3 cause dissociation of keratinocytes in canine pemphigus vulgaris and paraneoplastic pemphigus. 54 61
17350107 2007
41
Desmoglein versus non-desmoglein signaling in pemphigus acantholysis: characterization of novel signaling pathways downstream of pemphigus vulgaris antigens. 54 61
17344213 2007
42
Pemphigus vulgaris: recent advances in our understanding of its pathogenesis. 54 61
17452959 2007
43
Imaging and force spectroscopy on desmoglein 1 using atomic force microscopy reveal multivalent Ca(2+)-dependent, low-affinity trans-interaction. 54 61
17657525 2007
44
Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. 54 61
17263817 2007
45
Using desmoglein 1 and 3 enzyme-linked immunosorbent assay as an adjunct diagnostic tool for pemphigus. 54 61
17339147 2007
46
Changes in desmoglein 1 expression and subcellular localization in cultured keratinocytes subjected to anti-desmoglein 1 pemphigus autoimmunity. 54 61
17058228 2007
47
[Gene fragments cloned and immune recognition studied preliminarily for desmoglein 4 in pemphigus vulgaris]. 54 61
17294735 2007
48
Treatment of refractory pemphigus with the anti-CD20 monoclonal antibody (rituximab). 54 61
17460402 2007
49
Inhibition of Rho A activity causes pemphigus skin blistering. 54 61
17130286 2006
50
IgA pemphigus--occurrence of anti-desmocollin 1 and anti-desmoglein 1 antibody reactivity in an individual patient. 54 61
17176412 2006

Variations for Pemphigus

Expression for Pemphigus

Search GEO for disease gene expression data for Pemphigus.

Pathways for Pemphigus

Pathways related to Pemphigus according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.91 PPL JUP EVPL DSP DSG4 DSG3
2
Show member pathways
11.85 JUP DSP DSG2 DSC2
3
Show member pathways
11.82 PPL JUP EVPL DSP DSG4 DSG3
4 11.71 PLAUR JUP DSP CDH3
5
Show member pathways
11.62 DSP DSG3 DSG2 DSG1
6
Show member pathways
11.49 PPL EVPL DST DSP

GO Terms for Pemphigus

Cellular components related to Pemphigus according to GeneCards Suite gene sharing:

(show all 16)
# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 10.39 PPL PLAUR JUP HLA-DRB1 EVPL DST
2 integral component of membrane GO:0016021 10.34 PLAUR HLA-DRB1 DST DSG4 DSG3 DSG2
3 plasma membrane GO:0005886 10.25 PPL PLAUR JUP HLA-DRB1 DST DSP
4 extracellular exosome GO:0070062 10.18 PPL JUP HLA-DRB1 EVPL DSP DSG3
5 cell surface GO:0009986 9.96 PLAUR HLA-DRB1 DSG2 CDH3 ANXA9
6 cell-cell junction GO:0005911 9.96 JUP DSP DSG4 DSG3 DSG2 DSG1
7 desmosome GO:0030057 9.9 PPL JUP EVPL DSP DSG4 DSG3
8 intermediate filament GO:0005882 9.85 PPL JUP EVPL DST DSP
9 cell-cell adherens junction GO:0005913 9.72 JUP DSC2 CDH3
10 intercalated disc GO:0014704 9.71 JUP DSP DSG2 DSC2
11 ficolin-1-rich granule membrane GO:0101003 9.7 DSP DSG1 DSC1
12 cornified envelope GO:0001533 9.7 PPL JUP EVPL DSP DSG4 DSG3
13 lateral plasma membrane GO:0016328 9.69 JUP DSG2 DSG1
14 fascia adherens GO:0005916 9.54 JUP DSP
15 hemidesmosome GO:0030056 9.54 JUP DST COL17A1
16 cell junction GO:0030054 9.5 PPL PLAUR JUP EVPL DST DSP

Biological processes related to Pemphigus according to GeneCards Suite gene sharing:

(show all 18)
# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 10.02 JUP DST DSG4 DSG3 DSG2 DSG1
2 neutrophil degranulation GO:0043312 9.96 PLAUR JUP DSP DSG1 DSC1
3 cell-cell adhesion GO:0098609 9.9 JUP DSP DSG4 DSG3 DSG2 DSG1
4 homophilic cell adhesion via plasma membrane adhesion molecules GO:0007156 9.86 DSG4 DSG3 DSG2 DSG1 DSC3 DSC2
5 wound healing GO:0042060 9.85 PPL EVPL DST DSP CDH3
6 epidermis development GO:0008544 9.8 EVPL DSP COL17A1 ATP2C1
7 regulation of heart rate by cardiac conduction GO:0086091 9.78 JUP DSP DSG2 DSC2
8 intermediate filament cytoskeleton organization GO:0045104 9.73 PPL EVPL DST DSP
9 keratinocyte differentiation GO:0030216 9.72 EVPL DSP DSG4
10 regulation of ventricular cardiac muscle cell action potential GO:0098911 9.71 JUP DSP DSG2 DSC2
11 calcium-dependent cell-cell adhesion via plasma membrane cell adhesion molecules GO:0016339 9.7 DSG1 CDH3 ATP2C1
12 cornification GO:0070268 9.7 PPL JUP EVPL DSP DSG4 DSG3
13 adherens junction organization GO:0034332 9.69 JUP DSP CDH3
14 bundle of His cell-Purkinje myocyte adhesion involved in cell communication GO:0086073 9.67 JUP DSP DSG2 DSC2
15 fibrinolysis GO:0042730 9.58 PLAUR ANXA9
16 hemidesmosome assembly GO:0031581 9.57 DST COL17A1
17 desmosome organization GO:0002934 9.54 DSP DSG2
18 keratinization GO:0031424 9.4 PPL JUP EVPL DSP DSG4 DSG3

Molecular functions related to Pemphigus according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 calcium ion binding GO:0005509 9.65 DST DSG4 DSG3 DSG2 DSG1 DSC3
2 cadherin binding GO:0045296 9.62 PPL JUP EVPL CDH3
3 cell adhesion molecule binding GO:0050839 9.33 JUP DSP DSG2
4 gamma-catenin binding GO:0045295 9.26 DSG1 DSC3
5 cell adhesive protein binding involved in bundle of His cell-Purkinje myocyte communication GO:0086083 8.92 JUP DSP DSG2 DSC2

Sources for Pemphigus

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
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
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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