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MCID: PMP001
MIFTS: 55

Pemphigus

Categories: Cancer diseases, Genetic diseases, Immune diseases, Rare diseases, Skin diseases
Genes Tissues Related diseases Publications Pathways Symptoms & Phenotypes Drugs
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Aliases & Classifications for Pemphigus

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MalaCards integrated aliases for Pemphigus:

Name: Pemphigus 12 74 20 36 54 42 15 17 71 32

Classifications:

MalaCards categories:
Global: Rare diseases Genetic diseases Cancer diseases
Anatomical: Skin diseases Immune diseases
See all MalaCards categories (disease lists)
ICD10: 32


External Ids:

Disease Ontology 12 DOID:9182
KEGG 36 H01648
ICD9CM 34 694.4
MeSH 44 D010392
NCIt 50 C34909
SNOMED-CT 67 156355008
ICD10 32 L10 L10.9
UMLS 71 C0030807
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Summaries for Pemphigus

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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. The drugs Triamcinolone and Mycophenolic acid have been mentioned in the context of this disorder. Affiliated tissues include skin, t cells and b cells, and related phenotypes are Decreased viability and Decreased viability

Disease Ontology : 12 An autoimmune disease of skin and connective tissue that is characterized by blistering of the outer layer of the skin and mucous membranes (mouth, nose, throat, eyes, and genitals), causeing lesions and blisters that are easily ruptured.

GARD : 20 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 (/ˈpɛmfɪɡəs/ or /pɛmˈfaɪɡəs/) is a rare group of blistering autoimmune diseases that affect... more...

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Related Diseases for Pemphigus

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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 683)
# Related Disease Score Top Affiliating Genes
1 pemphigus erythematosus 32.9 DSP DSG3
2 pemphigus vegetans 32.8 PPL DSG1 DSC3
3 pemphigus foliaceus 32.4 PPL HLA-DRB1 EVPL DST DSG3 DSG2
4 pemphigoid 32.3 DST COL17A1
5 iga pemphigus 32.2 PPL EVPL DSG3 DSG1 DSC3 DSC1
6 benign chronic pemphigus 32.1 JUP DSP DSG4 DSG3 DSG1 DSC3
7 herpetiform pemphigus 32.1 DST DSG3 DSG1
8 epidermolysis bullosa, lethal acantholytic 31.8 JUP DSP
9 bullous pemphigoid 31.6 PPL HLA-DRB1 EVPL DST DSP DSG3
10 cicatricial pemphigoid 31.5 HLA-DRB1 DST COL17A1 CD79A
11 pemphigoid gestationis 31.4 HLA-DRB1 DST DSG3 DSG1 COL17A1
12 autoimmune disease 31.2 HLA-DRB1 DSG3 DSG1 COL17A1 CD79A
13 ritter's disease 31.0 DSP DSG4 DSG3 DSG2 DSG1 DSC3
14 thymoma 31.0 PPL EVPL DSP
15 follicular dendritic cell sarcoma 30.8 PPL EVPL DSP DSG3
16 lichen planus 30.8 DST DSG3 DSG1 COL17A1
17 bullous impetigo 30.8 DSG3 DSG1
18 paraneoplastic pemphigus 30.7 PPL HLA-DRB1 EVPL DST DSP DSG3
19 subcorneal pustular dermatosis 30.7 PPL EVPL DST DSP DSG3 DSG1
20 pemphigus vulgaris, familial 30.7 PPL PLAUR JUP HLA-DRB1 EVPL DST
21 keratosis 30.7 DSP DSG4 DSG2 DSG1
22 grover's disease 30.7 JUP DSP
23 linear iga disease 30.6 DST COL17A1 CD79A
24 impetigo 30.4 DSG4 DSG3 DSG2 DSG1 DSC3 DSC1
25 epidermolysis bullosa 30.3 DST DSP COL17A1
26 lichen planus pemphigoides 30.3 DST COL17A1
27 skin disease 30.2 DST DSP DSG4 DSG3 DSG2 DSG1
28 ocular cicatricial pemphigoid 30.1 HLA-DRB1 CD79A
29 pompholyx 29.8 DST COL17A1
30 epidermolysis bullosa acquisita 29.8 HLA-DRB1 DST COL17A1
31 pseudomembranous conjunctivitis 29.7 PPL EVPL DSP DSG3 DSG1
32 hypotrichosis 29.5 DSG4 DSG1 DSC3 CDH3
33 left bundle branch hemiblock 29.4 DSP DSG2 DSC2
34 darier-white disease 29.3 JUP DSP DSG4 DSG3 DSC3 DSC2
35 brugada syndrome 29.1 JUP DSP DSG2 DSC3 DSC2
36 bullous skin disease 28.9 PPL EVPL DST DSP DSG4 DSG3
37 pemphigus and fogo selvagem 11.4
38 pemphigus gestationis 11.2
39 superficial pemphigus 11.1
40 recessive dystrophic epidermolysis bullosa-generalized other 10.9
41 dermatitis herpetiformis 10.6
42 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 10.6
43 dermatitis 10.6
44 dermatitis herpetiformis, familial 10.6
45 thymoma, familial 10.5
46 myasthenia gravis 10.5
47 pustulosis of palm and sole 10.5
48 psoriasis 10.5
49 bronchiolitis 10.5
50 stomatitis 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
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Symptoms & Phenotypes for Pemphigus

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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 GR00055-A-1 9.62 PLAUR
2 Decreased viability GR00221-A-1 9.62 PLAUR
3 Decreased viability GR00221-A-2 9.62 PLAUR
4 Decreased viability GR00221-A-4 9.62 PLAUR
5 Decreased viability GR00249-S 9.62 ANXA9 ATP2C1 CD79A COL17A1 DSC3
6 Decreased viability GR00381-A-1 9.62 CD79A
7 Decreased viability GR00381-A-3 9.62 CD79A
8 Decreased viability GR00386-A-1 9.62 A2ML1 CD79A DSC3 EVPL HLA-DRB1
9 Decreased viability GR00402-S-2 9.62 ANXA9 DSG4 DSP EVPL

MGI Mouse Phenotypes related to Pemphigus:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 integument MP:0010771 9.4 ATP2C1 CDH3 DSC1 DSC3 DSG1 DSG2
Sources

Drugs & Therapeutics for Pemphigus

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

(show top 50) (show all 104)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Triamcinolone Approved, Vet_approved Phase 3 124-94-7 31307
2
Mycophenolic acid Approved Phase 3 24280-93-1 446541
3
rituximab Approved Phase 3 174722-31-7 10201696
4
Fexofenadine Approved, Investigational Phase 3 83799-24-0 3348
5
Ocrelizumab Approved, Investigational Phase 3 637334-45-3
6
Prednisolone phosphate Approved, Vet_approved Phase 3 302-25-0
7
Methylprednisolone Approved, Vet_approved Phase 3 83-43-2 6741
8
Prednisolone Approved, Vet_approved Phase 3 50-24-8 5755
9 Prednisolone acetate Approved, Vet_approved Phase 3 52-21-1
10
Methylprednisolone hemisuccinate Approved Phase 3 2921-57-5
11
Histamine Approved, Investigational Phase 3 51-45-6, 75614-87-8 774
12
Prednisone Approved, Vet_approved Phase 3 53-03-2 5865
13
ofatumumab Approved Phase 3 679818-59-8 6918251
14
Acetaminophen Approved Phase 3 103-90-2 1983
15
Cetirizine Approved Phase 3 83881-51-0 2678
16
Tyrosine Approved, Investigational, Nutraceutical Phase 3 60-18-4 6057
17
Prednisolone hemisuccinate Experimental Phase 3 2920-86-7
18 triamcinolone acetonide Phase 3
19 Triamcinolone hexacetonide Phase 3
20 Triamcinolone diacetate Phase 3
21 Antibiotics, Antitubercular Phase 3
22 Anti-Infective Agents Phase 3
23 Anti-Bacterial Agents Phase 3
24 Antitubercular Agents Phase 3
25 Antineoplastic Agents, Immunological Phase 3
26 Gastrointestinal Agents Phase 3
27 Antiemetics Phase 3
28 Protective Agents Phase 3
29 Paraproteins Phase 3
30 Neuroprotective Agents Phase 3
31 Myeloma Proteins Phase 3
32 Anti-Inflammatory Agents Phase 3
33 glucocorticoids Phase 3
34 Hormone Antagonists Phase 3
35 Hormones Phase 3
36 Antineoplastic Agents, Hormonal Phase 3
37 Antibodies, Monoclonal Phase 3
38 Methylprednisolone Acetate Phase 3
39 Histamine Antagonists Phase 3
40
Histamine Phosphate Phase 3 51-74-1 65513
41 Anti-Allergic Agents Phase 3
42 Neurotransmitter Agents Phase 3
43 Histamine H1 Antagonists Phase 3
44 Histamine H1 Antagonists, Non-Sedating Phase 3
45 Analgesics, Non-Narcotic Phase 3
46 Analgesics Phase 3
47 Antipyretics Phase 3
48
Lenograstim Approved, Investigational Phase 2 135968-09-1
49
Azathioprine Approved Phase 2 446-86-6 2265
50
Etanercept Approved, Investigational Phase 2 185243-69-0

Interventional clinical trials:

(show top 50) (show all 52)
# 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 Clinical and Immunological Long-term Follow-up of Patients With Pemphigus Included in the "RITUXIMAB 3" Trial With Medico-economic Evaluation of the Treatment. Unknown status NCT03790293 Phase 3 Rituximab;corticosteroids'therapy
3 EVALUATION CLINIQUE ET BIOLOGIQUE A LONG TERME DES PATIENTS ATTEINTS DE FORMES SEVERES DE PEMPHIGUS TRAITES PAR LE RITUXIMAB Completed NCT01299857 Phase 3 Rituximab
4 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)
5 Comparison Between Monoclonal Antibody CD20 Treatment (Rituximab (mabthéra))and General Corticotherapy Treatment in Patients With Pemphigus Completed NCT00784589 Phase 3 General Corticotherapy;Rituximab
6 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
7 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
8 Traitment of Patients Presenting Pemphigus With Anti CD20 (Mabthera). Completed NCT00213512 Phase 2, Phase 3 Mabthera
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 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
11 A Randomized, Double-Blinded, Placebo-Controlled Trial to Investigate the Efficacy, Safety, and Tolerability of Efgartigimod PH20 SC in Adult Patients With Pemphigus (Vulgaris or Foliaceus) Recruiting NCT04598451 Phase 3 prednisone
12 A Randomized, Double-Blind, Placebo-Controlled, Multicenter Trial to Evaluate the Efficacy and Safety of Oral BTK Inhibitor PRN1008 in Moderate to Severe Pemphigus Active, not recruiting NCT03762265 Phase 3 PRN1008 Oral Tablet;Placebo Oral Tablet
13 Danish Non-inferiority Study of Ocrelizumab and Rituximab in MS (DanNORMS): A Randomized Study Comparing the Efficacy of Ocrelizumab and Rituximab in Active Multiple Sclerosis Not yet recruiting NCT04688788 Phase 3 Rituximab;Ocrelizumab;Fexofenadine;Paracetamol;Methylprednisolone
14 An Open-Label, Multicenter, Follow-up Trial of ARGX-113-1904 to Evaluate the Safety, Tolerability, and Efficacy of Efgartigimod PH20 SC in Patients With Pemphigus Not yet recruiting NCT04598477 Phase 3 prednisone
15 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
16 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
17 Phase II Study of High-Dose Cyclophosphamide in Patients With Refractory Pemphigus Completed NCT00010413 Phase 2 cyclophosphamide;filgrastim
18 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
19 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 Completed NCT02704429 Phase 2 PRN1008
20 A Double-Blinded, Placebo-Controlled Study of Etanercept (Enbrel) in the Treatment of Pemphigus Vulgaris Completed NCT00135720 Phase 2 Enbrel (Etanercept)
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 A Phase 2 Open-Label Uncontrolled Pilot Study of KC706 in Patients With Stable, Active Pemphigus Vulgaris Completed NCT00606749 Phase 2 KC706
23 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
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) Completed NCT03334058 Phase 2 ARGX-113
25 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 Recruiting NCT04023149 Phase 2 recombinant human interleukin-2 (rhIL-2);placebo
26 The Use of IVIG in Combination With Rituximab VS Rituximab as the First Line Treatment of Pemphigus Recruiting NCT04400994 Phase 2 Rituximab
27 Dexamethasone Solution and Dexamethasone in Mucolox™ for the Treatment of Oral Inflammatory Ulcerative Diseases Recruiting NCT04540133 Phase 2 dexamethasone 0.5mg/5ml solution;dexamethasone 0.5mg/5ml solution in Mucolox™
28 A Randomized, Partial-blind, Placebo-controlled Trial Evaluating the Efficacy, Safety, Pharmacokinetics and Pharmacodynamics of VAY736 in the Treatment of Patients With Pemphigus Vulgaris Terminated NCT01930175 Phase 2 VAY736;Placebo
29 A Prospective Randomized Placebo-Controlled Clinical Trial of Dapsone as a Glucocorticoid-Sparing Agent in Maintenance Phase Pemphigus Vulgaris Terminated NCT00429533 Phase 2 Dapsone
30 Phase 2 Randomized Trial of IVIg With or Without Cyclophosphamide in Pemphigus Terminated NCT00483119 Phase 2 intravenous immunoglobulin;cyclophosphamide
31 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 ALXN1830
32 A Phase 2 Dose-Escalation Study of the Safety and Tolerability of INCB050465 in Participants With Pemphigus Vulgaris Withdrawn NCT03780166 Phase 2 Parsaclisib
33 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
34 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
35 A Phase 1, Open-label, Safety and Dosing Study of Autologous Desmoglein 3 Chimeric Autoantibody Receptor T Cells (DSG3-CAART) in Subjects With Active, Anti-DSG3, Mucosal-dominant Pemphigus Vulgaris Recruiting NCT04422912 Phase 1
36 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) Active, not recruiting NCT03239470 Phase 1
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 Identification of Vulnerability Factors in the Course of Pemphigus Patients - SHS Pemphigus Clinical Trial Unknown status NCT02237313
39 Serum Free Fatty Acids Desaturase Activity Indices in Sera of Patients on Long-term High-dose Corticosteroid Treatment Unknown status NCT02872584 Prednisone
40 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
41 An Open Randomized Comparative Trial of Four Different Dressings for Cutaneous Coverage in Pemphigus and Pemphigoid Unknown status NCT02365675
42 Serum IL-21 Levels in Patients With Pemphigus Vulgaris Unknown status NCT03177213
43 Assessment of the Enrichment of Rare Coding Genetic Variants in Patients Affected by Neutrophil-Mediated Inflammatory Dermatoses Unknown status NCT01952275
44 Research Registry for Inherited Disorders of Keratinization Unknown status NCT00074685
45 Expression of hSPCA1 and Ultrastructural Analysis of the Skin Before and After Laser Therapy in Hailey-Hailey Disease Unknown status NCT03849989
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 Phenotypic and Functional Characterisation of Human B-cell Response in Pemphigus and Application to Other Auto-immune Diseases Recruiting NCT04117529
49 Prevalence and Clinical Severity of Autoimmune Blistering Diseases Recruiting NCT02753777
50 Prospective Analysis of the Th17 Cellular Response in Pemphigus Vulgaris Patients Treated With Corticosteroids and Optional Adjuvants Not yet recruiting NCT04096222

Search NIH Clinical Center for Pemphigus

Inferred drug relations via UMLS 71 / NDF-RT 51 :


Auranofin
Aurothioglucose
Chloroquine
Chloroquine hydrochloride
chloroquine phosphate
Potassium aminobenzoate
Sources

Genetic Tests for Pemphigus

About this section
Sources

Anatomical Context for Pemphigus

About this section

MalaCards organs/tissues related to Pemphigus:

40
Skin, T Cells, B Cells, Thyroid, Cervix, Eye, Breast
Sources

Publications for Pemphigus

About this section

Articles related to Pemphigus:

(show top 50) (show all 8905)
# Title Authors PMID Year
1
Predominant Stroma-Rich Feature in Hyaline Vascular Variant of Castleman Disease Is Associated With Paraneoplastic Pemphigus. 61 42
32459333 2020
2
Clinical characteristics, mortality, and prognostic factors for bullous pemphigoid in a Thai population. 42
33120819 2020
3
Recent progresses and perspectives in autoimmune bullous diseases. 42
32272983 2020
4
C3d immunohistochemistry on formalin-fixed tissue is a valuable tool in the diagnosis of bullous pemphigoid of the skin. 61 54
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. 61 54
20163452 2010
6
p38MAPK signaling and desmoglein-3 internalization are linked events in pemphigus acantholysis. 61 54
20093368 2010
7
A case of pemphigus herpetiformis with absence of antibodies to desmogleins 1 and 3. 61 54
20050294 2010
8
Development of ELISA for the specific determination of autoantibodies against envoplakin and periplakin in paraneoplastic pemphigus. 54 61
19737550 2009
9
Autoantibodies against desmocollins in European patients with pemphigus. 61 54
19456767 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
Subcorneal pustular dermatosis-type IgA pemphigus with autoantibodies to desmocollins 1, 2, and 3. 54 61
19841404 2009
12
Oral pemphigus. 61 54
19893475 2009
13
Dermatoses affecting desmosomes in animals: a mechanistic review of acantholytic blistering skin diseases. 54 61
20178467 2009
14
Detection of anti-envoplakin and anti-periplakin autoantibodies by ELISA in patients with paraneoplastic pemphigus. 54 61
18820940 2009
15
[IgA pemphigus accompanying multiple myeloma has disappeared following the treatment with bortezomib (Velcade), cyclophosphamide and dexamethasone. Case study and literature review]. 61 54
19947244 2009
16
Desmocollin 3-mediated binding is crucial for keratinocyte cohesion and is impaired in pemphigus. 61 54
19717567 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. 61 54
19467379 2009
19
An 'n-of-1' placebo-controlled crossover trial of intravenous immunoglobulin as adjuvant therapy in refractory pemphigus vulgaris. 61 54
19222460 2009
20
The value of desmoglein 1 and 3 antibody ELISA testing in patients with pemphigus. 61 54
19451505 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. 61 54
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
Discohesive malignant melanoma simulating a bullous dermatoses. 61 54
19208079 2009
25
High-dose pemphigus antibodies against linear epitopes of desmoglein 3 (Dsg3) can induce acantholysis and depletion of Dsg3 from keratinocytes. 61 54
19200441 2009
26
Pemphigus: the promises of peptide immunotherapy. 54 61
19874216 2009
27
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. 61 54
20049340 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. 61 54
18641320 2008
31
E-cadherin is an additional immunological target for pemphigus autoantibodies. 61 54
18219275 2008
32
Immunoglobulin A pemphigus associated with immunoglobulin A gammopathy and lung cancer. 61 54
18578711 2008
33
Evaluating efficacy of plasmapheresis for patients with pemphigus using desmoglein enzyme-linked immunosorbent assay. 61 54
18241273 2008
34
Enzyme-linked immunosorbent assay in autoimmune blistering diseases: preliminary experience of the Dermatology Department of Cagliari. 61 54
18833046 2008
35
A perspective of pemphigus from bedside and laboratory-bench. 61 54
18094947 2007
36
Beyond steric hindrance: the role of adhesion signaling pathways in the pathogenesis of pemphigus. 61 54
17574391 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. 61 54
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
Coexistence of IgA antibodies to desmogleins 1 and 3 in pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. 61 54
17263817 2007
43
Pemphigus vulgaris: recent advances in our understanding of its pathogenesis. 54 61
17452959 2007
44
Imaging and force spectroscopy on desmoglein 1 using atomic force microscopy reveal multivalent Ca(2+)-dependent, low-affinity trans-interaction. 61 54
17657525 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. 61 54
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). 61 54
17460402 2007
49
IgA pemphigus--occurrence of anti-desmocollin 1 and anti-desmoglein 1 antibody reactivity in an individual patient. 54 61
17176412 2006
50
Inhibition of Rho A activity causes pemphigus skin blistering. 61 54
17130286 2006
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Variations for Pemphigus

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Expression for Pemphigus

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Search GEO for disease gene expression data for Pemphigus.
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Pathways for Pemphigus

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Pathways related to Pemphigus according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Developmental Biology 65
Show member pathways
 12.91 PPL JUP EVPL DSP DSG4 DSG3
2
Arrhythmogenic right ventricular cardiomyopathy 36 1
11.85 JUP DSP DSG2 DSC2
3
Keratinization 65
Show member pathways
 11.82 PPL JUP EVPL DSP DSG4 DSG3
4
Adhesion 4
11.71 PLAUR JUP DSP CDH3
5
Apoptotic cleavage of cellular proteins 65
Show member pathways
 11.62 DSP DSG3 DSG2 DSG1
6
Cytoskeleton remodeling Neurofilaments 23
Show member pathways
 11.49 PPL EVPL DST DSP
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GO Terms for Pemphigus

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

(show all 14)
# Name GO ID Score Top Affiliating Genes
1 membrane GO:0016020 10.41 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 10.28 PPL PLAUR JUP HLA-DRB1 DST DSP
4 extracellular exosome GO:0070062 10.18 PPL JUP HLA-DRB1 EVPL DSP DSG3
5 cell-cell junction GO:0005911 9.96 JUP DSP DSG4 DSG3 DSG2 DSG1
6 desmosome GO:0030057 9.9 PPL JUP EVPL DSP DSG4 DSG3
7 intermediate filament GO:0005882 9.85 PPL JUP HLA-DRB1 EVPL DST DSP
8 intercalated disc GO:0014704 9.73 JUP DSP DSG2 DSC2
9 lateral plasma membrane GO:0016328 9.7 JUP DSG2 DSG1
10 cornified envelope GO:0001533 9.7 PPL JUP EVPL DSP DSG4 DSG3
11 ficolin-1-rich granule membrane GO:0101003 9.69 DSP DSG1 DSC1
12 hemidesmosome GO:0030056 9.58 JUP DST COL17A1
13 fascia adherens GO:0005916 9.54 JUP DSP
14 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 16)
# Name GO ID Score Top Affiliating Genes
1 cell adhesion GO:0007155 10.07 JUP DST DSG4 DSG3 DSG2 DSG1
2 neutrophil degranulation GO:0043312 9.95 PLAUR JUP DSP DSG1 DSC1
3 cell-cell adhesion GO:0098609 9.9 JUP DSP DSG4 DSG3 DSG2 DSG1
4 epidermis development GO:0008544 9.88 HLA-DRB1 EVPL DSP COL17A1 ATP2C1
5 homophilic cell adhesion via plasma membrane adhesion molecules GO:0007156 9.86 DSG4 DSG3 DSG2 DSG1 DSC3 DSC2
6 wound healing GO:0042060 9.85 PPL EVPL DST DSP CDH3
7 regulation of heart rate by cardiac conduction GO:0086091 9.8 JUP DSP DSG2 DSC2
8 intermediate filament cytoskeleton organization GO:0045104 9.73 PPL EVPL DST DSP
9 keratinocyte differentiation GO:0030216 9.71 EVPL DSP DSG4
10 regulation of ventricular cardiac muscle cell action potential GO:0098911 9.71 JUP DSP DSG2 DSC2
11 cornification GO:0070268 9.7 PPL JUP EVPL DSP DSG4 DSG3
12 adherens junction organization GO:0034332 9.69 JUP DSP CDH3
13 bundle of His cell-Purkinje myocyte adhesion involved in cell communication GO:0086073 9.62 JUP DSP DSG2 DSC2
14 hemidesmosome assembly GO:0031581 9.56 DST COL17A1
15 desmosome organization GO:0002934 9.52 DSP DSG2
16 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 cadherin binding GO:0045296 9.71 PPL JUP EVPL CDH3
2 structural molecule activity GO:0005198 9.55 PPL JUP EVPL DST DSP
3 structural constituent of cytoskeleton GO:0005200 9.54 PPL HLA-DRB1 DSP
4 cell adhesion molecule binding GO:0050839 9.43 JUP DSP DSG2
5 calcium ion binding GO:0005509 9.36 DST DSG4 DSG3 DSG2 DSG1 DSC3
6 gamma-catenin binding GO:0045295 9.32 DSG1 DSC3
7 cell adhesive protein binding involved in bundle of His cell-Purkinje myocyte communication GO:0086083 9.26 JUP DSP DSG2 DSC2
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Sources for Pemphigus

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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