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pemphigus-vulgaris
Pemphigus Vulgaris
Pemphigus vulgaris (PV) is an IgG4-mediated autoimmune blistering disease targeting Dsg3 (mucous membranes) and Dsg1 (skin); suprabasal acantholysis. Rituximab (PEMPHIX: 90% vs 28% CR; FDA Jun 2018) and efgartigimod (ADHERE-SC; FDA Oct 2023) are approved therapies.
Entry Metadata
| Field | Value |
|---|---|
| ID | pemphigus-vulgaris |
| Name | Pemphigus Vulgaris |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
connects-toDesmoglein 3
connects-toImmunoglobulin G
connects-toCd20
connects-toFcrn
connects-toB Cell
connects-toEgfr
connects-toComplement C3
connects-toSkin
connects-toPlasma Cell
connects-toT Helper Cell
connects-toMyasthenia Gravis
connects-toSystemic Lupus Erythematosus
connects-toDermatomyositis
connects-toRegulatory T Cell
connects-toRheumatoid Arthritis
connects-toType 1 Diabetes
connects-toDendritic Cell
connects-toIl 4
connects-toEye
connects-toImmune System
connects-toIntegumentary System
connects-toDigestive System
connects-toMhc Class Ii
connects-toComplement C5
connects-toIl 6
connects-toCalcium
connects-toCortisol
connects-toMast Cell
connects-toNf Kb
connects-toIl 17A
connects-toT Cytotoxic Cell
connects-toSodium
connects-toThymus
connects-toNeutrophil
connects-toElectron
connects-toPhoton
connects-toAcetylcholine
connects-toCollagen
connects-toOsteoporosis
connects-toLung
connects-toAntibody
connects-toStaphylococcus Aureus
connects-toReproductive System
connects-toBaff
connects-toCll
connects-toVitamin D
connects-toBtk
connects-toSepsis
connects-toThyroid
connects-toTnf Alpha
connects-toSjogrens Syndrome
connects-toVenous Thromboembolism
connects-toStat3
connects-toPneumocystis Jirovecii
connects-toCandida Albicans
connects-toType 2 Diabetes
connects-toAspergillus Fumigatus
connects-toMajor Depressive Disorder
connects-toVaricella Zoster Virus
connects-toHepatitis B Virus
connects-toGeneralized Anxiety Disorder
connects-toWound Healing
connects-toEndocrine System
connects-toMusculoskeletal System
connects-toRespiratory System
connects-toCardiovascular System
connects-toLymphatic System
connects-toCorticosteroids
connects-toRenal System
connects-toHerpesvirus
connects-toCancer Chemotherapy
connects-toCidp
connects-toNmo
connects-toGerminal Center
connects-toPsoriasis
connects-toSystemic Sclerosis
connects-toIntestinal Epithelium
connects-toAlveolus
connects-toFollicular Lymphoma
connects-toWaldenstrom Macroglobulinemia
connects-toAnca Vasculitis
connects-toCovid 19 Disease
connects-toCdh1
connects-toIfn Gamma
connects-toIl 1B
connects-toIl 10
connects-toNlrp3 Inflammasome
connects-toMacrophage
connects-toSrc Kinase
connects-toCaspase 3
connects-toCtla 4
connects-toJak1 2
connects-toIl 2
connects-toBcl 2
connects-toIl 13
connects-toTgf Beta
connects-toIl 23
connects-toErk1 2
connects-toAkt
connects-toIl 5
connects-toIl 12
connects-toMyd88
connects-toPik3Ca
connects-toMtor
connects-toGalectin 3
connects-toSmad4
connects-toStat1
connects-toCgas Sting
connects-toFoxo
connects-toS100A8 A9
connects-toGsk 3B
connects-toPerforin
connects-toAutophagy
connects-toDnmt3A
connects-toCcl2
connects-toAmpk
connects-toCcr5
connects-toCalcineurin
connects-toCxcl12
connects-toIl 33
connects-toType I Interferon
connects-toGlucocorticoid Receptor
connects-toIge
connects-toThyroid Hormones
connects-toSecretory Iga
connects-toMu Opioid Receptor
connects-toEstrogen
connects-toNitric Oxide
connects-toVegf
connects-toXanthine Oxidase
connects-toProstaglandins
connects-toHistamine
connects-toZinc
connects-toIron
connects-toPotassium
connects-toMagnesium
connects-toLeptin
connects-toAdiponectin
connects-toResistin
connects-toNatural Killer Cell
connects-toIl 31
connects-toTslp
connects-toAtopic Dermatitis
connects-toPrurigo Nodularis
connects-toPeriostin
connects-toC5Ar1
connects-toSelenium
connects-toSubstance P
connects-toFactor H
connects-toC1 Esterase Inhibitor
connects-toTransferrin
connects-toBradykinin
connects-toErythropoietin
connects-toProlactin
connects-toMelatonin
connects-toOxytocin
connects-toTestosterone
connects-toSerotonin
connects-toVasopressin
connects-toCalcium
connects-toIodine
connects-toCopper
connects-toPhosphorus
connects-toChloride
connects-toSulfur
connects-toNitrogen
connects-toOxygen
connects-toCarbon
connects-toHydrogen
connects-toPd 1
connects-toGlp 1
connects-toAngiotensin Ii
connects-toRankl
connects-toWnt Beta Catenin
connects-toFibronectin
connects-toSmad4
connects-toIl 2
connects-toNotch
connects-toIgf 1
connects-toActivin A
connects-toCgrp
connects-toCalcitonin
connects-toInsulin Receptor
connects-toAldosterone
connects-toAndrogen Receptor
connects-toNorepinephrine
connects-toAdrenomedullin
connects-toBdnf
connects-toOsteopontin
connects-toFgfr
connects-toEpinephrine
connects-toRenin
connects-toMyostatin
Sources
- Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017;389(10083):2031-2040. · PubMed 28342637
- Murrell DF, Sprecher E, Maho-Vaillant M, et al. Efgartigimod alfa and hyaluronidase-qvfc in pemphigus vulgaris. N Engl J Med. 2024;390(5):419-430. · PubMed 38294978
- Amagai M, Tsunoda K, Zillikens D, Nagai T, Nishikawa T. The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol. 1999;40(2 Pt 1):167-170. · PubMed 10025737