Pemphigus vulgaris
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Summary
Pemphigus vulgaris (MONDO:0008219) is a disease with 2 cohort genes (11 GWAS associations across 4 studies) and 39 clinical trials. Top therapeutic interventions include efgartigimod alfa, mycophenolate mofetil, and azathioprine.
At a glance
- Prevalence: 1-5 / 10 000 (Europe) [Orphanet-validated]
- Cohort genes: 2
- GWAS associations: 11
- Phenotypes (HPO): 20
- Clinical trials: 39
Clinical features
Epidemiology
Prevalence records
8 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 18 | Europe | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.13 | France | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.7 | United Kingdom | Validated |
| Annual incidence | <1 / 1 000 000 | 0.076 | Finland | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.47 | Bulgaria | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.42 | United States | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.25 | Tunisia | Validated |
| Point prevalence | 1-9 / 100 000 | 9.482 | Germany | Validated |
Signs & symptoms
Clinical features (HPO)
20 HPO clinical features (Orphanet curated; top 20 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0000987 | Atypical scarring of skin | Very frequent (80-99%) |
| HP:0001025 | Urticaria | Very frequent (80-99%) |
| HP:0001824 | Weight loss | Very frequent (80-99%) |
| HP:0002719 | Recurrent infections | Very frequent (80-99%) |
| HP:0002960 | Autoimmunity | Very frequent (80-99%) |
| HP:0008872 | Feeding difficulties in infancy | Very frequent (80-99%) |
| HP:0031446 | Erosion of oral mucosa | Very frequent (80-99%) |
| HP:0100792 | Acantholysis | Very frequent (80-99%) |
| HP:0100838 | Recurrent cutaneous abscess formation | Very frequent (80-99%) |
| HP:0200097 | Oral mucosal blisters | Very frequent (80-99%) |
| HP:0000716 | Depression | Frequent (30-79%) |
| HP:0000739 | Anxiety | Frequent (30-79%) |
| HP:0008066 | Abnormal blistering of the skin | Frequent (30-79%) |
| HP:0011968 | Feeding difficulties | Frequent (30-79%) |
| HP:0034194 | Suprabasal cleavage | Frequent (30-79%) |
| HP:4000013 | Anti-desmoglein-1 antibody positivity | Frequent (30-79%) |
| HP:4000014 | Anti-desmoglein-3 antibody positivity | Frequent (30-79%) |
| HP:0002293 | Alopecia of scalp | Occasional (5-29%) |
| HP:0012531 | Pain | Occasional (5-29%) |
| HP:0030351 | Urticarial plaque | Very rare (<1-4%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | pemphigus vulgaris |
| Mondo ID | MONDO:0008219 |
| EFO | EFO:0004719 |
| MeSH | C536645 |
| OMIM | 169610 |
| Orphanet | 704 |
| DOID | DOID:0060851 |
| ICD-10-CM | L10.0 |
| ICD-11 | 278358681 |
| NCIT | C34910 |
| SNOMED CT | 49420001 |
| UMLS | C0030809 |
| MedGen | 10621 |
| GARD | 0007355 |
| MedDRA | 10052802 |
| Is cancer (heuristic) | no |
Data availability: 11 GWAS associations (4 studies) · 18 cell lines.
Disease family
An umbrella term covering 1 Mondo subtype.
Classification path: disease › human disease › disease by body system or component › integumentary system disorder › skin disorder › dermatitis › autoimmune bullous skin disease › pemphigus › pemphigus vulgaris
Related subtypes (5): pemphigoid gestationis, Hailey-Hailey disease, herpetiform pemphigus, pemphigus erythematosus, pemphigus foliaceus
Subtypes (1): pemphigus vegetans
Genetics & variants
GWAS landscape
11 GWAS associations across 4 studies. Top hits map to 3 distinct genes (as reported by GWAS).
Top associations by p-value
| rsID | p-value | Gene | Risk allele | Odds ratio |
|---|---|---|---|---|
| rs70993900 | 1e-45 | HLA-DRB1 - HLA-DQA1 | ? | 18.57 |
| HLA-DRB1*14:04 | 2e-38 | ? | 6.28 | |
| rs28483633 | 3e-35 | HLA-DQA1 - HLA-DQB1 | ? | 5.86 |
| rs9469220 | 4e-19 | HLA-DQB1 - MTCO3P1 | G | 3.36 |
| rs7454108 | 3e-12 | MTCO3P1 - HLA-DQB3 | ? | 3.25 |
| rs11218708 | 3e-08 | RNU6-592P - GLULP3 | A | 1.54 |
| rs1074532 | 1e-06 | EPHA5-AS1 - RNU2-40P | A | 1.81 |
| rs117814792 | 2e-06 | LINC02161 - MIR3660 | A | 3.12 |
| rs1051336 | 3e-06 | HLA-DRA | ? | 3.03 |
| rs9262122 | 5e-06 | PPP1R10 | ? | 2.73 |
| rs16837853 | 8e-06 | THSD7B | G | 2 |
Top studies (by case count)
| Study | Lead author | Year | Cases | Controls | Title |
|---|---|---|---|---|---|
| GCST008068 | Zhang S | 2018 | 255 | 0 | Subtype-Specific Inherited Predisposition to Pemphigus in Chinese Population. |
| GCST006584 | Gao J | 2018 | 240 | 1,031 | Association study and fine-mapping major histocompatibility complex analysis of pemphigus vulgaris in a Han Chinese population. |
| GCST006521 | Sun Y | 2018 | 101 | 0 | Investigation of the Predisposing Factor of Pemphigus and its clinical subtype through a Genome-wide association and next generation sequence analysis. |
| GCST001452 | Sarig O | 2012 | 100 | 0 | Population-specific association between a polymorphic variant in ST18, encoding a pro-apoptotic molecule, and pemphigus vulgaris. |
Variant details and genetic-evidence tiers
Tier distribution (top 50 variants)
| Tier | Variants |
|---|---|
| Tier 1: coding | 0 |
| Tier 2: splice/UTR | 1 |
| Tier 3: regulatory | 2 |
| Tier 4: intronic/intergenic | 8 |
MAF distribution
| Bucket | Variants |
|---|---|
| common (>=0.05) | 9 |
| low_freq (0.01-0.05) | 0 |
| rare (<0.01) | 0 |
| unknown | 2 |
Functional consequences
| Consequence | Count |
|---|---|
| intergenic_variant | 4 |
| intron_variant | 3 |
| unknown | 1 |
| TF_binding_site_variant | 1 |
| regulatory_region_variant | 1 |
| 3_prime_UTR_variant | 1 |
Top variants
| rsID | Chr | Pos | Alleles | MAF | Consequence | Gene | p-value | Tier |
|---|---|---|---|---|---|---|---|---|
| rs70993900 | 6 | 32618381 | AC>A,ACC | 0.09 | intergenic_variant | HLA-DRB1 - HLA-DQA1 | 1e-45 | Tier 4: intronic/intergenic |
| HLA-DRB1*14:04 | 2e-38 | Tier 4: intronic/intergenic | ||||||
| rs28483633 | 6 | 32657097 | C>A | 0.05 | TF_binding_site_variant | HLA-DQA1 - HLA-DQB1 | 3e-35 | Tier 3: regulatory |
| rs9469220 | 6 | 32690533 | G>A,T | 0.33 | intergenic_variant | HLA-DQB1 - MTCO3P1 | 4e-19 | Tier 4: intronic/intergenic |
| rs7454108 | 6 | 32713706 | T>C | 0.05 | intergenic_variant | MTCO3P1 - HLA-DQB3 | 3e-12 | Tier 4: intronic/intergenic |
| rs11218708 | 11 | 122601355 | G>A | 0.21 | regulatory_region_variant | RNU6-592P - GLULP3 | 3e-08 | Tier 3: regulatory |
| rs1074532 | 4 | 65791342 | A>C,G | 0.05 | intergenic_variant | EPHA5-AS1 - RNU2-40P | 1e-06 | Tier 4: intronic/intergenic |
| rs117814792 | 5 | 90014044 | G>A | intron_variant | LINC02161 - MIR3660 | 2e-06 | Tier 4: intronic/intergenic | |
| rs1051336 | 6 | 32444815 | G>A,C | 0.05 | 3_prime_UTR_variant | HLA-DRA | 3e-06 | Tier 2: splice/UTR |
| rs9262122 | 6 | 30606065 | T>C | 0.05 | intron_variant | PPP1R10 | 5e-06 | Tier 4: intronic/intergenic |
| rs16837853 | 2 | 137014290 | A>C,G | 0.05 | intron_variant | THSD7B | 8e-06 | Tier 4: intronic/intergenic |
Genes & proteins
Mendelian disease overlap and somatic drivers
GenCC: 0 · Orphanet: 14 · OMIM-shared: 0 · Dual-evidence (GWAS+Mendelian): 0
Orphanet rare-disease linkage (cohort genes)
| Gene | Orphanet ID | Rare disease |
|---|---|---|
| HLA-DRB1 | Orphanet:2073 | Narcolepsy type 1 |
| HLA-DRB1 | Orphanet:220393 | Diffuse cutaneous systemic sclerosis |
| HLA-DRB1 | Orphanet:220402 | Limited cutaneous systemic sclerosis |
| HLA-DRB1 | Orphanet:220407 | Limited systemic sclerosis |
| HLA-DRB1 | Orphanet:3437 | Vogt-Koyanagi-Harada disease |
| HLA-DRB1 | Orphanet:397 | Giant cell arteritis |
| HLA-DRB1 | Orphanet:477738 | Pediatric multiple sclerosis |
| HLA-DRB1 | Orphanet:536 | Systemic lupus erythematosus |
| HLA-DRB1 | Orphanet:545 | Follicular lymphoma |
| HLA-DRB1 | Orphanet:703 | Bullous pemphigoid |
| HLA-DRB1 | Orphanet:747 | Autoimmune pulmonary alveolar proteinosis |
| HLA-DRB1 | Orphanet:797 | Sarcoidosis |
| HLA-DRB1 | Orphanet:83465 | Narcolepsy type 2 |
| HLA-DRB1 | Orphanet:85414 | Systemic-onset juvenile idiopathic arthritis |
Cohort genes → proteins
2 cohort genes, 2 distinct canonical proteins.
Evidence partition
| Subset | Genes |
|---|---|
| gwas_only | 2 |
Cohort genes (full)
| Symbol | HGNC | Ensembl | UniProt | Name | Evidence |
|---|---|---|---|---|---|
| UBASH3B | HGNC:29884 | ENSG00000154127 | Q8TF42 | Ubiquitin-associated and SH3 domain-containing protein B | gwas |
| HLA-DRB1 | HGNC:4948 | ENSG00000196126 | P01911 | HLA class II histocompatibility antigen, DRB1 beta chain | gwas |
Cohort function summary
Lead sentence per gene, UniProt-curated.
| Symbol | Protein name | Function (lead sentence) |
|---|---|---|
| UBASH3B | Ubiquitin-associated and SH3 domain-containing protein B | Interferes with CBL-mediated down-regulation and degradation of receptor-type tyrosine kinases. |
| HLA-DRB1 | HLA class II histocompatibility antigen, DRB1 beta chain | A beta chain of antigen-presenting major histocompatibility complex class II (MHCII) molecule. |
Protein-family classification
Druggable: 2 · Difficult: 0 · Unknown: 0 · Druggable fraction: 1.0
Family distribution
Cohort families vs a genome-wide background (hypergeometric, BH-FDR; fold = observed/expected). Counts kept; sorted by enrichment, so the catch-all Other/Unknown bucket no longer leads.
| Family | Genes | Fold | FDR |
|---|---|---|---|
| Phosphatase | 1 | 42.0× | 0.047 |
| Antibody/Immunoglobulin | 1 | 14.6× | 0.067 |
Per-gene assignment
| Symbol | Family | Druggable? | EC | InterPro (top 3) |
|---|---|---|---|---|
| UBASH3B | Phosphatase | yes | SH3_domain, UBA-like_sf, His_Pase_superF_clade-1 | |
| HLA-DRB1 | Antibody/Immunoglobulin | yes | MHC_II_b_N, Ig/MHC_CS, Ig_C1-set |
Expression context
Cohort genes with no expression data: 0.
2 cohort genes are a single-cell marker in ≥1 SCXA experiment.
Breadth distribution (Bgee present_calls)
| Bucket | Genes |
|---|---|
| narrow (1-5 tissues) | 0 |
| moderate (6-20) | 0 |
| broad (>20) | 2 |
| unknown | 0 |
Top tissues across cohort
| Tissue | Cohort genes |
|---|---|
| cerebellar cortex | 1 |
| cerebellum | 1 |
| pons | 1 |
| granulocyte | 1 |
| right lung | 1 |
| vermiform appendix | 1 |
Per-gene tissue summary (top 30)
| Symbol | Bgee breadth | FANTOM5 breadth | SCXA | Top tissues |
|---|---|---|---|---|
| UBASH3B | 212 | ubiquitous | marker | pons, cerebellum, cerebellar cortex |
| HLA-DRB1 | 131 | tissue_specific | marker | vermiform appendix, granulocyte, right lung |
Protein interactions among cohort
Intra-cohort edges: 0.
Hub genes (top 10 by interactor count)
| Symbol | Interactor count |
|---|---|
| HLA-DRB1 | 3,448 |
| UBASH3B | 1,820 |
Structural data
PDB: 2 · AlphaFold-only: 0 · No structure: 0
Cohort genes with PDB structures (top 30)
| Symbol | UniProt | PDB entries |
|---|---|---|
| HLA-DRB1 | P01911 | 108 |
| UBASH3B | Q8TF42 | 6 |
Function
Pathway analysis
Distinct Reactome pathways touched by cohort: 7. Enrichment computed across 2 evidence-associated genes (1 with Reactome annotation).
Pathways by enrichment
Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 1 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.
| Pathway | Cohort genes | Fold | FDR | Sample cohort genes |
|---|---|---|---|---|
| Translocation of ZAP-70 to Immunological synapse | 1 | 634.4× | 0.004 | HLA-DRB1 |
| Phosphorylation of CD3 and TCR zeta chains | 1 | 543.8× | 0.004 | HLA-DRB1 |
| Co-inhibition by PD-1 | 1 | 519.1× | 0.004 | HLA-DRB1 |
| Generation of second messenger molecules | 1 | 346.1× | 0.005 | HLA-DRB1 |
| Downstream TCR signaling | 1 | 128.3× | 0.009 | HLA-DRB1 |
| Interferon gamma signaling | 1 | 125.5× | 0.009 | HLA-DRB1 |
| MHC class II antigen presentation | 1 | 89.2× | 0.011 | HLA-DRB1 |
GO biological processes by enrichment
Over-representation of cohort genes vs the genome-wide background (hypergeometric test, Benjamini-Hochberg FDR; fold = observed/expected over 2 annotated cohort genes). Counts and members are kept as ground-truth; sorted by enrichment.
| GO term | Cohort genes | Fold | FDR | Sample cohort genes |
|---|---|---|---|---|
| regulation of interleukin-4 production | 1 | 8426.0× | 0.003 | HLA-DRB1 |
| antigen processing and presentation of endogenous peptide antigen via MHC class II | 1 | 4213.0× | 0.003 | HLA-DRB1 |
| regulation of interleukin-10 production | 1 | 4213.0× | 0.003 | HLA-DRB1 |
| myeloid dendritic cell antigen processing and presentation | 1 | 2808.7× | 0.003 | HLA-DRB1 |
| regulation of T-helper cell differentiation | 1 | 2106.5× | 0.003 | HLA-DRB1 |
| positive regulation of CD4-positive, alpha-beta T cell activation | 1 | 2106.5× | 0.003 | HLA-DRB1 |
| positive regulation of CD4-positive, CD25-positive, alpha-beta regulatory T cell differentiation | 1 | 1685.2× | 0.003 | HLA-DRB1 |
| positive regulation of T cell mediated immune response to tumor cell | 1 | 1203.7× | 0.004 | HLA-DRB1 |
| T-helper 1 type immune response | 1 | 936.2× | 0.004 | HLA-DRB1 |
| positive regulation of memory T cell differentiation | 1 | 936.2× | 0.004 | HLA-DRB1 |
| positive regulation of monocyte differentiation | 1 | 766.0× | 0.004 | HLA-DRB1 |
| regulation of osteoclast differentiation | 1 | 766.0× | 0.004 | UBASH3B |
| detection of bacterium | 1 | 702.2× | 0.004 | HLA-DRB1 |
| negative regulation of platelet aggregation | 1 | 702.2× | 0.004 | UBASH3B |
| collagen-activated tyrosine kinase receptor signaling pathway | 1 | 648.1× | 0.004 | UBASH3B |
| peptide antigen assembly with MHC class II protein complex | 1 | 526.6× | 0.004 | HLA-DRB1 |
| negative regulation of bone resorption | 1 | 495.6× | 0.004 | UBASH3B |
| inflammatory response to antigenic stimulus | 1 | 468.1× | 0.004 | HLA-DRB1 |
| regulation of release of sequestered calcium ion into cytosol | 1 | 468.1× | 0.004 | UBASH3B |
| protein tetramerization | 1 | 312.1× | 0.006 | HLA-DRB1 |
| negative regulation of inflammatory response to antigenic stimulus | 1 | 300.9× | 0.006 | HLA-DRB1 |
| antigen processing and presentation of exogenous peptide antigen via MHC class II | 1 | 271.8× | 0.006 | HLA-DRB1 |
| negative regulation of osteoclast differentiation | 1 | 271.8× | 0.006 | UBASH3B |
| positive regulation of T cell mediated cytotoxicity | 1 | 255.3× | 0.006 | HLA-DRB1 |
| positive regulation of immune response | 1 | 240.7× | 0.006 | HLA-DRB1 |
| macrophage differentiation | 1 | 234.1× | 0.006 | HLA-DRB1 |
| positive regulation of T cell activation | 1 | 221.7× | 0.006 | HLA-DRB1 |
| negative regulation of type II interferon production | 1 | 191.5× | 0.007 | HLA-DRB1 |
| negative regulation of signal transduction | 1 | 187.2× | 0.007 | UBASH3B |
| positive regulation of insulin secretion involved in cellular response to glucose stimulus | 1 | 187.2× | 0.007 | HLA-DRB1 |
Therapeutics
Drugs indicated for this disease
3 approved, 4 in late-stage (phase 3) trials. Disease-direct ChEMBL indications, not inferred from the associated-gene cohort below.
| Drug | Development status |
|---|---|
| Dexamethasone | Approved (phase 4) |
| Prednisone | Approved (phase 4) |
| Rituximab | Approved (phase 4) |
| Mycophenolate Mofetil | Phase 3 (in late-stage trials) |
| Ofatumumab | Phase 3 (in late-stage trials) |
| Rilzabrutinib | Phase 3 (in late-stage trials) |
| Triamcinolone Acetonide | Phase 3 (in late-stage trials) |
Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Azathioprine, Dapsone, Etanercept, Human Immunoglobulin G, Ianalumab, Parsaclisib.
Drug target analysis
Approved (phase 4): 0 · Phase ≥3: 1 · Phased (≥1): 1 · Undrugged: 1
Druggability breadth: 2 of 2 evidence-associated genes (100%) have a ChEMBL target (buckets above are over the deeply-mined display cohort).
Top cohort targets by molecule count
| Symbol | Molecules | Max phase |
|---|---|---|
| UBASH3B | 1 | 3 |
| HLA-DRB1 | 0 | 0 |
Drugs targeting cohort genes (top 30)
| Molecule | Max phase | Targets in cohort |
|---|---|---|
| REBAMIPIDE | 3 | UBASH3B |
Bioactivity and enzyme data
Enzyme cohort genes (≥1 EC): 0.
Cohort genes with ChEMBL bioactivity (full, sorted by assay count)
| Symbol | Assays | Type breakdown |
|---|---|---|
| HLA-DRB1 | 17 | Binding:17 |
| UBASH3B | 10 | Binding:10 |
Pharmacogenomics
Cohort genes with a PharmGKB record: 2; with CPIC/DPWG dosing guidelines: 0.
No cohort gene has a CPIC/DPWG genotype-guided dosing guideline (PharmGKB).
Chemical tractability of cohort targets
1 approved/phased compounds have measured bioactivity against a cohort gene (and aren’t yet in disease-level trials). This is a research / tractability signal, NOT a therapeutic recommendation — a bioactivity row often reflects off-target or screening binding (e.g. promiscuous kinase inhibitors against a cohort kinase), implying no disease mechanism.
| Compound | Max phase | Cohort target (bioactivity) |
|---|---|---|
| REBAMIPIDE | 3 | UBASH3B |
Druggability pyramid
Cohort genes binned by druggability tier (high → low):
| Tier | Definition | Genes | Symbols |
|---|---|---|---|
| A | Approved (phase 4 drug) | 0 | |
| B | Phased (≥1) drug, not yet approved | 1 | UBASH3B |
| C | Druggable family + PDB, no drug | 1 | HLA-DRB1 |
| D | Druggable family + AlphaFold only, no drug | 0 | |
| E | Difficult family or no structure, no drug | 0 |
Undrugged target profiles
1 cohort genes are undrugged. Ranked by ‘starting-point quality’ (assay depth + drugged-partner adjacency).
| Symbol | ChEMBL assays | Drugged partners (top 3) |
|---|---|---|
| HLA-DRB1 | 17 | — |
Clinical trials & evidence
Clinical trials
Clinical trials: 39.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 13 |
| Not specified | 12 |
| PHASE3 | 6 |
| PHASE1/PHASE2 | 3 |
| PHASE1 | 3 |
| PHASE4 | 2 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT05303272 | PHASE4 | UNKNOWN | A Study to Evaluate Efficacy and Safety of Abatacept in Participants of Pemphigus Vulgaris (PV) |
| NCT06846255 | PHASE4 | COMPLETED | Comparing DCP and DAP for Pemphigus Vulgaris |
| NCT00683930 | PHASE3 | COMPLETED | A Study to Assess the Effect of CellCept (Mycophenolate Mofetil) and Reduced Corticosteroids in Patients With Active Pemphigus Vulgaris (PV) |
| NCT01920477 | PHASE3 | TERMINATED | Efficacy and Safety of Ofatumumab in Treatment of Pemphigus Vulgaris |
| NCT02383589 | PHASE3 | COMPLETED | A Study to Evaluate the Efficacy and Safety of Rituximab Versus Mycophenolate Mofetil (MMF) in Participants With Pemphigus Vulgaris (PV) |
| NCT04598451 | PHASE3 | COMPLETED | A Study to Assess the Efficacy and Safety of a Subcutaneous Formulation of Efgartigimod PH20 SC in Adults With Pemphigus (Vulgaris or Foliaceus) |
| NCT04598477 | PHASE3 | TERMINATED | A Study to Assess the Long-term Safety and Efficacy of a Subcutaneous Formulation of Efgartigimod PH20 SC in Adults With Pemphigus (Vulgaris or Foliaceus) |
| NCT05594472 | PHASE3 | UNKNOWN | Ozonated Olive Oil in Treatment of Pemphigus Vulgaris and Bullous Pemphigoid |
| NCT04422912 | PHASE1/PHASE2 | RECRUITING | A Phase 1/2, Open-label, Safety and Dosing Study of Autologous CART Cells (Desmoglein 3 Chimeric Autoantibody Receptor T Cells [DSG3-CAART] or CD19-specific Chimeric Antigen Receptor T Cells [CABA-201]) in Subjects With Active, Pemphigus Vulgaris (RESET-PV) |
| NCT06663943 | PHASE1/PHASE2 | NOT_YET_RECRUITING | A Randomized Study on Pemphigus Treatment With Humanized CD38 Antibody CM313. |
| NCT00135720 | PHASE2 | COMPLETED | Study of Etanercept (Enbrel) in the Treatment of Pemphigus Vulgaris |
| NCT00429533 | PHASE2 | TERMINATED | Efficacy of Dapsone as a Steroid Sparing Agent in Pemphigus Vulgaris |
| NCT00483119 | PHASE2 | TERMINATED | Randomized Trial of IVIg With or Without Cyclophosphamide in Pemphigus |
| NCT00606749 | PHASE2 | COMPLETED | Use of KC706 for the Treatment of Pemphigus Vulgaris |
| NCT00626678 | PHASE2 | COMPLETED | Azathioprine Versus Placebo in Pemphigus Vulgaris Treated With Prednisolone |
| NCT01930175 | PHASE2 | TERMINATED | Study of Efficacy and Safety of VAY736 in Patients With Pemphigus Vulgaris |
| NCT02704429 | PHASE2 | COMPLETED | A Study of PRN1008 in Adult Patients With Pemphigus Vulgaris |
| NCT03075904 | PHASE1/PHASE2 | TERMINATED | A Safety and Dose-Finding Study of SYNT001 in Subjects With Pemphigus (Vulgaris or Foliaceus) |
| NCT03334058 | PHASE2 | COMPLETED | A Study to Evaluate the Safety, PD, PK and Efficacy of ARGX-113 in Patients With Pemphigus |
| NCT03780166 | PHASE2 | WITHDRAWN | A Study of the Safety and Tolerability of INCB050465 in Pemphigus Vulgaris |
| NCT04023149 | PHASE2 | UNKNOWN | Efficacy of Interleukin-2 Gargle in the Treatment of Oral Mucosa Lesion in Pemphigus Vulgaris |
| NCT04540133 | PHASE2 | COMPLETED | Dexamethasone Solution and Dexamethasone in Mucolox™ |
| NCT05000216 | PHASE2 | TERMINATED | COVID-19 Booster Vaccine in Autoimmune Disease Non-Responders |
| NCT07461103 | PHASE2 | COMPLETED | Effect of Topical Insulin on Healing Rate of Pemphigus Lesions |
| NCT06581562 | PHASE1 | RECRUITING | Open-label Single-Center Study to Evaluate the Safety and Efficacy of Combining Rituximab and AB-101 in B-cell Associated Autoimmune Diseases. |
| NCT00063752 | PHASE1 | COMPLETED | Safety Study of PI-0824 to Treat Pemphigus Vulgaris |
| NCT03239470 | PHASE1 | TERMINATED | Polyclonal Regulatory T Cells (PolyTregs) for Pemphigus |
| NCT02753777 | Not specified | RECRUITING | Autoimmune Blistering Diseases Study |
| NCT05635266 | Not specified | RECRUITING | Tissue Repository Providing Annotated Biospecimens for Approved Investigator-directed Biomedical Research Initiatives |
| NCT06863454 | Not specified | NOT_YET_RECRUITING | Trichoscopy as a Monitoring Tool for Activity and Remission in Pemphigus Vulgaris: A Clinical Study Supported by Immunological Evaluation. |
| NCT06971172 | Not specified | NOT_YET_RECRUITING | Low-level Laser Therapy on Oral Pemphigus Vulgaris Patients. |
| NCT07384221 | Not specified | RECRUITING | Periodontal Status and Disease Severity in Pemphigus Vulgaris Patients |
| NCT03177213 | Not specified | UNKNOWN | Serum IL-21 Levels in Patients With Pemphigus Vulgaris |
| NCT04096222 | Not specified | UNKNOWN | Comparative Analysis of the Th17 Cellular Response in Active and Inactive Pemphigus Vulgaris Patients |
| NCT04117529 | Not specified | UNKNOWN | Phenotypic and Functional Characterisation of Human B-cell Response in Pemphigus |
| NCT05338112 | Not specified | UNKNOWN | Role of Tzanck Smear in Determining Pemphigus Vulgaris Disease Activity |
| NCT06167408 | Not specified | COMPLETED | Identifying Factors Influencing In-Hospital Relapse in Pemphigus Patients |
| NCT06654817 | Not specified | COMPLETED | Efficacy and Safety Evaluation of Anti-CD20 Monoclonal Antibody Combined with Azathioprine and Corticosteroids in the Treatment of Pemphigus Vulgaris |
| NCT07428915 | Not specified | COMPLETED | Evaluating Legit.Health Plus Support for Improving Diagnosis of Generalized Pustular Psoriasis and Other Skin Conditions Among Primary Care Physicians and Dermatologists |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| EFGARTIGIMOD ALFA | 4 | 3 |
| MYCOPHENOLATE MOFETIL | 4 | 2 |
| AZATHIOPRINE | 4 | 1 |
| DAPSONE | 4 | 1 |
| ELASOMERAN | 4 | 1 |
| GENTAMICIN SULFATE | 4 | 1 |
| OFATUMUMAB | 4 | 1 |
| TOZINAMERAN | 4 | 1 |
| IANALUMAB | 3 | 1 |
| PARSACLISIB | 3 | 1 |
| RILZABRUTINIB | 3 | 1 |
| AB-101 | 2 | 1 |
| ITX-5061 | 2 | 1 |
| LUMROTATUG | 2 | 1 |
| ORILANOLIMAB | 2 | 1 |
| PI-0824 | 1 | 1 |
Related Atlas pages
- Cohort genes: UBASH3B, HLA-DRB1
- Drugs: Efgartigimod Alfa, Mycophenolate Mofetil, Azathioprine, Dapsone, Elasomeran, Gentamicin, Ofatumumab, Tozinameran, Ianalumab, Parsaclisib, Rilzabrutinib