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pemphigus vulgaris - Clinical Profile, Treatments & Trials | Kisho | Kisho

Rare Disease

pemphigus vulgaris

MONDO:0008219

Also known as:familial pemphigus vulgarispemphigus vulgaris, familial

Find Clinical TrialsTrials

Overview

Pemphigus is a group of chronic autoimmune skin diseases characterized by blister formations on the outer layer of the skin and the mucous membranes. Three clinical forms have been characterized, of which pemphigus vulgaris is the most frequent (75%).

GeneticsAutosomal dominant

Treatments1 FDA approved

Clinical Trials11 registered · 5 recruiting

Clinical Presentation

Built from Human Phenotype Ontology (HPO) phenotype annotations and GeneReviews clinical descriptions. Features represent findings documented across known cases — not an exhaustive list of possible symptoms.

Features include: Autoimmunity, Autoimmune antibody positivity, Abnormal blistering of the skin, and Oral mucosal blisters.

Organ System	Phenotype Count	Example Features
Blood and immune system	2	Autoimmunity, Autoimmune antibody positivity

Diagnosis

Biomarker and diagnostic research for pemphigus vulgaris has been reported in the published literature.

Treatment

Compiled from FDA-approved drug records, orphan drug designations, and FDA Adverse Event Reporting System (FAERS) safety data. GeneReviews management guidance is included where available.

1 FDA-approved treatment is available for pemphigus vulgaris, including rituximab (Rituxan, approved 2002). An additional 5 compounds hold orphan drug designation.

Approved Therapies

Brand Name	Generic Name	Mechanism	Approved	Market Status

Prognosis

Estimated prevalence: 1-5 in 10,000 (Uncommon).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

11 clinical trials registered, 5 recruiting. Interventions under study include drug therapy, other interventions, medical devices, and biologic therapy. Pipeline includes 3 PHASE1, 1 EARLY_PHASE1, 3 NA. Research is sponsored by a mix of industry and academic institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT02753777](https://clinicaltrials.gov/study/NCT02753777)

Data assembled from 7 of 12 sources · Last updated Sep 18, 2026, 10:50 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

OMIM

Online Mendelian Inheritance in Man

OMIM:169610

Orphanet

European rare disease database

Orphanet:704

GARD

Genetic and Rare Diseases Info Center

GARD:7355

Questions for Your Care Team

→What are the benefits and potential side effects of Rituxan for managing pemphigus vulgaris?
→Are any of the 11 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Brand Name	Generic Name	Sponsor	Designated	Exclusivity End	Designation Status
Autologous Desmoglein 3 (DSG3) Chimeric Autoantibody Receptor-directed (CAAR) T cells	Autologous Desmoglein 3 (DSG3) Chimeric Autoantibody Receptor-directed (CAAR) T cells	Cabaletta Bio, Inc.	2020	—	Designated
Small Molecule Bruton's Agammaglobulinemia Tyrosine Kinase (BKT) Inhibitor	Small Molecule Bruton's Agammaglobulinemia Tyrosine Kinase (BKT) Inhibitor	Principia Biopharma Inc.	2017	—	Withdrawn
(S)-2-(1-((6-amino-5-cyanopyrimidin-4-yl)amino)ethyl)-4-oxo-3-phenyl-3,4-dihydropyrrolo[2,1-f][1,2,4]triazine-5-carbonitrile	(S)-2-(1-((6-amino-5-cyanopyrimidin-4-yl)amino)ethyl)-4-oxo-3-phenyl-3,4-dihydropyrrolo[2,1-f][1,2,4]triazine-5-carbonitrile	Almirall S.A.	2015	—	Withdrawn
Mycophenolate mofetil	Mycophenolate mofetil	Hoffman-La Roche, Inc.	2006	—	Withdrawn
Desmoglein 3 synthetic peptide (PI-0824)	Desmoglein 3 synthetic peptide (PI-0824)	Peptimmune, Inc.	2004	—	Designated

Research Type	Count	% of Total
Research summaries	59	27%
Laboratory research	40	18%
Patient case studies	36	17%
Clinical study results	27	12%
Disease patterns and progression	24	11%
Testing and diagnosis research	12	6%
Other research	11	5%
New treatment approaches	8	4%

Kisho

pemphigus vulgaris

Overview

Clinical Presentation

Diagnosis

Treatment

Approved Therapies

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Orphan Drug Designations

Clinical Trials

Recent Publications

Latest News & Developments

Protocol for evaluating the effects of the intelligent multimodal symptom assessment and response system in patients with pemphigus vulgaris: a randomised controlled trial.

Genetic and immunological determinants of Pemphigus vulgaris: integrative analysis of HLA-DRB1 and FCGR2B variants.

Postsurgical pemphigus vulgaris following thyroidectomy: a case report and review of the literature.

pemphigus vulgaris

Overview

Clinical Presentation

Diagnosis

Treatment

Approved Therapies

Prognosis

Research Landscape

Medical References

External Resources

OMIM

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Explore clinical features

Orphan Drug Designations

Clinical Trials

Recent Publications

Latest News & Developments

Protocol for evaluating the effects of the intelligent multimodal symptom assessment and response system in patients with pemphigus vulgaris: a randomised controlled trial.

Genetic and immunological determinants of Pemphigus vulgaris: integrative analysis of HLA-DRB1 and FCGR2B variants.

Postsurgical pemphigus vulgaris following thyroidectomy: a case report and review of the literature.