Emerging treatment options for the management of pemphigus vulgaris

References

• BystrynJCRudolphJLPemphigusLancet20053669479617315993235
   PubMed Web of Science ®Google Scholar
• HertlMJedlickovaHKarpatiSPemphigus. S2 Guideline for diagnosis and treatment – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV)J Eur Acad Dermatol Venereol201529340541425338479
   PubMed Web of Science ®Google Scholar
• BystrynJCSteinmanNMThe adjuvant therapy of pemphigus. An updateArch Dermatol199613222032128629830
   PubMedGoogle Scholar
• KridinKZelber-SagiSBergmanRPemphigus vulgaris and Pemphigus foliaceus: differences in epidemiology and mortalityActa Derm Venereol20179791095109928536732
   PubMed Web of Science ®Google Scholar
• KridinKSagiSZBergmanRMortality and cause of death in patients with pemphigusActa Derm Venereol201797560761128093595
   PubMed Web of Science ®Google Scholar
• MurrellDFDickSAhmedARConsensus statement on definitions of disease, end points, and therapeutic response for pemphigusJ Am Acad Dermatol20085861043104618339444
   PubMed Web of Science ®Google Scholar
• MartinLKWerthVPVillaneuvaEVMurrellDFA systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceusJ Am Acad Dermatol201164590390821353333
   PubMed Web of Science ®Google Scholar
• HarmanKEBrownDExtonLSBritish Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017Br J Dermatol201717751170120129192996
   PubMed Web of Science ®Google Scholar
• AlmugairenNHospitalVBedaneCAssessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroidsJ Am Acad Dermatol201369458358823850258
   PubMed Web of Science ®Google Scholar
• GregoriouSEfthymiouOStefanakiCRigopoulosDManagement of pemphigus vulgaris: challenges and solutionsClin Cosmet Investig Dermatol20158521527
   PubMed Web of Science ®Google Scholar
• DickSEWerthVPPemphigus: a treatment updateAutoimmunity200639759159917101503
   PubMed Web of Science ®Google Scholar
• MentinkLFMackenzieMWTóthGGRandomized controlled trial of adjuvant oral dexamethasone pulse therapy in pemphigus vulgaris: PEMPULS trialArch Dermatol2006142557057616702494
   PubMedGoogle Scholar
• TóthGGWesterlakenBOEildersMLaseurMJonkmanMFUgesDRDexamethasone pharmacokinetics after high-dose oral therapy for pemphigusAnn Pharmacother20023661108110912058710
   PubMed Web of Science ®Google Scholar
• AhmedARMoyRDeath in pemphigusJ Am Acad Dermatol1982722212287130483
   PubMed Web of Science ®Google Scholar
• FernandesNCPerezMTreatment of pemphigus vulgaris and pemphigus foliaceus: experience with 71 patients over a 20 year periodRev Inst Med Trop Sao Paulo2001431333611246281
   PubMedGoogle Scholar
• FauciASDaleDCBalowJEGlucocorticosteroid therapy: mechanisms of action and clinical considerationsAnn Intern Med1976843304315769625
   PubMed Web of Science ®Google Scholar
• BoumpasDTChrousosGPWilderRLCuppsTRBalowJEGlucocorticoid therapy for immune-mediated diseases: basic and clinical correlatesAnn Intern Med199311912119812088239251
   PubMed Web of Science ®Google Scholar
• BarnesPJCorticosteroid effects on cell signallingEur Respir J200627241342616452600
   PubMed Web of Science ®Google Scholar
• NelsonHSLeungDYMBloomJWUpdate on glucocorticoid action and resistanceJ Allergy Clin Immunol2003111132212532089
   PubMed Web of Science ®Google Scholar
• LiuDAhmetAWardLA practical guide to the monitoring and management of the complications of systemic corticosteroid therapyAllergy Asthma Clin Immunol2013913023947590
   PubMed Web of Science ®Google Scholar
• RatnamKVPhayKLTanCKPemphigus therapy with oral prednisolone regimens A 5-year studyInt J Dermatol19902953633672361795
   PubMed Web of Science ®Google Scholar
• FemianoFGombosFScullyCPemphigus vulgaris with oral involvement: evaluation of two different systemic corticosteroid therapeutic protocolsJ Eur Acad Dermatol Venereol200216435335612224691
   PubMed Web of Science ®Google Scholar
• Chams-DavatchiCMortazavizadehADaneshpazhoohMRandomized double blind trial of prednisolone and azathioprine, vs. prednisolone and placebo, in the treatment of pemphigus vulgarisJ Eur Acad Dermatol Venereol201327101285129223062214
   PubMed Web of Science ®Google Scholar
• BeissertSWerfelTFrielingUA comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigusArch Dermatol2006142111447145417116835
   PubMedGoogle Scholar
• BeissertSMimouniDKanwarAJSolomonsNKaliaVAnhaltGJTreating pemphigus vulgaris with prednisone and mycophenolate mofetil: a multicenter, randomized, placebo-controlled trialJ Invest Dermatol201013082041204820410913
   PubMed Web of Science ®Google Scholar
• IoannidesDApallaZLazaridouERigopoulosDEvaluation of mycophenolate mofetil as a steroid-sparing agent in pemphigus: a randomized, prospective studyJ Eur Acad Dermatol Venereol201226785586021752101
   PubMed Web of Science ®Google Scholar
• Chams-DavatchiCEsmailiNDaneshpazhoohMRandomized controlled open-label trial of four treatment regimens for pemphigus vulgarisJ Am Acad Dermatol200757462262817583373
   PubMed Web of Science ®Google Scholar
• AtzmonyLHodakELeshemYAThe role of adjuvant therapy in pemphigus: a systematic review and meta-analysisJ Am Acad Dermatol201573226427126088689
   PubMed Web of Science ®Google Scholar
• SandbornWJState-of-the-art: immunosuppression and biologic therapyDig Dis201028353654220926883
   PubMed Web of Science ®Google Scholar
• SchiavoALPucaRVRuoccoVRuoccoEAdjuvant drugs in autoimmune bullous diseases, efficacy versus safety: facts and controversiesClin Dermatol201028333734320541689
   PubMed Web of Science ®Google Scholar
• MeurerMImmunosuppressive therapy for autoimmune bullous diseasesClin Dermatol2012301788322137230
   PubMed Web of Science ®Google Scholar
• MaltzmanJSKoretzkyGAAzathioprine: old drug, new actionsJ Clin Invest200311181122112412697731
   PubMed Web of Science ®Google Scholar
• AbererWWolff-SchreinerECStinglGWolffKAzathioprine in the treatment of pemphigus vulgaris: a long-term follow-upJ Am Acad Dermatol1987163 Pt 15275333819096
   PubMed Web of Science ®Google Scholar
• AnsteyAVWakelinSReynoldsNJAzathioprine: guidelines for prescribing azathioprine in dermatologyCoxNHEnglishJSBritish Association of Dermatologists’ Management GuidelinesChichesterWiley-Blackwell2011219228
   Google Scholar
• TavakolpourSCurrent and future treatment options for pemphigus: is it time to move towards more effective treatments?Int Immunopharmacol20175313314229107213
   PubMed Web of Science ®Google Scholar
• MeggittSJAnsteyAVMohd MustapaMFReynoldsNJWakelinSBritish Association of Dermatologists’ guidelines for the safe and effective prescribing of azathioprine 2011Br J Dermatol2011165471173421950502
   PubMed Web of Science ®Google Scholar
• KasperkiewiczMSchmidtEZillikensDCurrent therapy of the pemphigus groupClin Dermatol2012301849422137231
   PubMed Web of Science ®Google Scholar
• MabroukDGürcanHMKeskinDBChristenWGAhmedARAssociation between cancer and immunosuppressive therapy – analysis of selected studies in pemphigus and pemphigoidAnn Pharmacother201044111770177620923942
   PubMed Web of Science ®Google Scholar
• KwongYLAuWYLiangRHAcute myeloid leukemia after azathioprine treatment for autoimmune diseases: association with -7/7q-Cancer Genet Cytogenet1998104294979666800
   PubMedGoogle Scholar
• TavakolpourSMirsafaeiHSDelshadSManagement of pemphigus disease in pregnancyAm J Reprod Immunol2017771
   Web of Science ®Google Scholar
• ChaidemenosGApallaZKoussidouTPapagarifallouIIoannidesDHigh dose oral prednisone vs. prednisone plus azathioprine for the treatment of oral pemphigus: a retrospective, bi-centre, comparative studyJ Eur Acad Dermatol Venereol201125220621020569289
   PubMed Web of Science ®Google Scholar
• AllisonACMechanisms of action of mycophenolate mofetilLupus200514Suppl 1s2s815803924
   PubMed Web of Science ®Google Scholar
• OlejarzWBrykDZapolska-DownarDMycophenolate mofetil – a new atheropreventive drug?Acta Pol Pharm201471335336125265813
   PubMed Web of Science ®Google Scholar
• NousariHCSragovichAKimyai-AsadiAOrlinskyDAnhaltGJMycophenolate mofetil in autoimmune and inflammatory skin disordersJ Am Acad Dermatol1999402 Pt I26526810025760
   PubMed Web of Science ®Google Scholar
• OrvisAKWessonSKBrezaTSChurchAAMitchellCLWatkinsSWMycophenolate mofetil in dermatologyJ Am Acad Dermatol200960218319919150270
   PubMed Web of Science ®Google Scholar
• EnsleyRDBristowMROlsenSLThe use of mycophenolate mofetil (RS-61443) in human heart transplant recipientsTransplantation199356175828333071
   PubMed Web of Science ®Google Scholar
• MimouniDAnhaltGJCumminsDLKoubaDJThorneJENousariHCTreatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetilArch Dermatol2003139673974212810504
   PubMedGoogle Scholar
• EmingRNagelAWolff-FrankeSPodstawaEDebusDHertlMRituximab exerts a dual effect in pemphigus vulgarisJ Invest Dermatol2008128122850285818563178
   PubMed Web of Science ®Google Scholar
• ZambrunoGBorradoriLRituximab immunotherapy in pemphigus: therapeutic effects beyond B-cell depletionJ Invest Dermatol2008128122745274718997839
   PubMed Web of Science ®Google Scholar
• MouquetHMusettePGougeonMLB-cell depletion immunotherapy in pemphigus: effects on cellular and humoral immune responsesJ Invest Dermatol2008128122859286918563177
   PubMed Web of Science ®Google Scholar
• KimJDanielBSWerthVPJolyPMurrellDFThe treatment of pemphigus vulgaris and pemphigus foliaceusMurrellDFBlistering Diseases: Clinical Features, Pathogenesis, TreatmentBerlinSpringer2015523531
   Google Scholar
• JolyPMaho-VaillantMProst-SquarcioniCFrench study group on autoimmune bullous skin diseasesFirst-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 trialLancet2017389100832031204028342637
   PubMed Web of Science ®Google Scholar
• SchmidtEBröckerEBGoebelerMRituximab in treatment-resistant autoimmune blistering skin disordersClin Rev Allergy Immunol2008341566418270859
   PubMed Web of Science ®Google Scholar
• HertlMZillikensDBorradoriLRecommendations for the use of rituximab (anti-CD20 antibody) in the treatment of autoimmune bullous skin diseasesJ Dtsch Dermatol Ges200865366373 German [with English abstract]18201220
   PubMed Web of Science ®Google Scholar
• ZakkaLRShettySSAhmedARRituximab in the treatment of pemphigus vulgarisDermatol Ther (Heidelb)2012211723205339
   PubMedGoogle Scholar
• LapeyreHJolyPRituximab: mabtheraAnn Dermatol Venereol20071341296897118166919
   PubMed Web of Science ®Google Scholar
• AhmedARSpigelmanZCavaciniLAPosnerMRTreatment of pemphigus vulgaris with rituximab and intravenous immune globulinN Engl J Med2006355171772177917065638
   PubMed Web of Science ®Google Scholar
• JolyPMouquetHRoujeauJCA single cycle of rituximab for the treatment of severe pemphigusN Engl J Med2007357654555217687130
   PubMed Web of Science ®Google Scholar
• ColliouNPicardDCaillotFLong-term remissions of severe pemphigus after rituximab therapy are associated with prolonged failure of desmoglein B cell responseSci Transl Med20135175175ra30
   PubMed Web of Science ®Google Scholar
• CianchiniGLupiFMasiniCCoronaRPudduPDe PitàOTherapy with rituximab for autoimmune pemphigus: results from a single-center observational study on 42 cases with long-term follow-upJ Am Acad Dermatol201267461762222243765
   PubMed Web of Science ®Google Scholar
• Hoffmann-La RocheA study to evaluate the efficacy and safety of rituximab versus mycophenolate mofetil (MMF) in participants with pemphigus vulgaris (PV) Available from: https://clinicaltrials.gov/ct2/show/study/NCT02383589?cond=pemphigus&draw=2&rank=11&view=results. NLM identifier: NCT02383589Accessed February 23, 2018
   Google Scholar
• ShraddhaUpretyComparative study of rituximab versus combination of rituximab and intravenous cyclophosphamide in severe pemphigus Available from: https://clinicaltrials.gov/ct2/show/NCT01974518?cond=pemphigus&draw=5&rank=3. NLM identifier: NCT01974518Accessed February 24, 2018
   Google Scholar
• SchmidtEGoebelerMZillikensDRituximab in severe pemphigusAnn N Y Acad Sci2009117368369119758216
   PubMed Web of Science ®Google Scholar
• RosenbachMMurrellDFBystrynJCReliability and convergent validity of two outcome instruments for pemphigusJ Invest Dermatol2009129102404241019357707
   PubMed Web of Science ®Google Scholar
• AwadAStüveOCyclophosphamide in multiple sclerosis: scientific rationale, history and novel treatment paradigmsTher Adv Neurol Disord200926357368
   Google Scholar
• AkhtarSJHasanMUTreatment of pemphigus: a local experienceJ Pak Med Assoc1998481030030410087750
   PubMedGoogle Scholar
• RoseEWeverSZillikenDLinseRHausteinUFBröckerEBIntravenous dexamethasone-cyclophosphamide pulse therapy in comparison with oral methylprednisolone-azathioprine therapy in patients with pemphigus: results of a multicenter prospectively randomized studyJ Dtsch Dermatol Ges20053320020616372814
   PubMedGoogle Scholar
• ChrysomallisFIoannidesDTeknetzisAPanagiotidouDMinasATreatment of oral pemphigus vulgarisInt J Dermatol199433118038077822089
   PubMedGoogle Scholar
• SharmaVKKhandpurSEvaluation of cyclophosphamide pulse therapy as an adjuvant to oral corticosteroid in the management of pemphigus vulgarisClin Exp Dermatol201338665966423837939
   PubMed Web of Science ®Google Scholar
• SethyPKKhandpurSSharmaVKRandomized open comparative trial of dexamethasone-cyclophosphamide pulse and daily oral cyclophosphamide versus cyclophosphamide pulse and daily oral prednisolone in pemphigus vulgarisIndian J Dermatol Venereol Leprol200975547648219736426
   PubMed Web of Science ®Google Scholar
• ParmarNVKanwarAJMinzRWAssessment of the therapeutic benefit of dexamethasone cyclophosphamide pulse versus only oral cyclophosphamide in phase II of the dexamethasone cyclophosphamide pulse therapy: a preliminary prospective randomized controlled studyIndian J Dermatol Venereol Leprol2013791707623254732
   PubMed Web of Science ®Google Scholar
• HartungHPAdvances in the understanding of the mechanism of action of IVIgJ Neurol2008255Suppl 336
   PubMed Web of Science ®Google Scholar
• AoyamaYWhat’s new in i.v. immunoglobulin therapy and pemphigus: high-dose i.v. immunoglobulin therapy and its mode of action for treatment of pemphigusJ Dermatol201037323924520507387
   PubMed Web of Science ®Google Scholar
• PrinsCGelfandEWFrenchLEIntravenous immunoglobulin: properties, mode of action and practical use in dermatologyActa Derm Venereol200787320621817533485
   PubMed Web of Science ®Google Scholar
• GreenMGBystrynJCEffect of intravenous immunoglobulin therapy on serum levels of IgG1 and IgG4 antidesmoglein 1 and antidesmoglein 3 antibodies in pemphigus vulgarisArch Dermatol2008144121621162419075146
   PubMedGoogle Scholar
• MimouniDBlankMAshkenaziLProtective effect of intravenous immunoglobulin (IVIG) in an experimental model of pemphigus vulgarisClin Exp Immunol2005142342643216297153
   PubMed Web of Science ®Google Scholar
• AmagaiMPemphigusBologniaJLJorizzoJLSchafferJVDermatology3rd edMadridElsevier2012461474
   Google Scholar
• ArnoldDFBurtonJShineBWojnarowskaFMisbahSAAn “n-of-1” placebo-controlled crossover trial of intravenous immunoglobulin as adjuvant therapy in refractory pemphigus vulgarisBr J Dermatol200916051098110219222460
   PubMed Web of Science ®Google Scholar
• AmagaiMIkedaSShimizuHPemphigus Study GroupA randomized double-blind trial of intravenous immunoglobulin for pemphigusJ Am Acad Dermatol200960459560319293008
   PubMed Web of Science ®Google Scholar
• BystrynJCJiaoDNatowSTreatment of pemphigus with intravenous immunoglobulinJ Am Acad Dermatol200247335836312196744
   PubMed Web of Science ®Google Scholar
• FerraraGZumlaAMaeurerMIntravenous immunoglobulin (IVIg) for refractory and difficult-to-treat infectionsAm J Med2012125101036.e1e8
   Web of Science ®Google Scholar
• SheehanDJLesherJLJrDeep venous thrombosis after high-dose intravenous immunoglobulin in the treatment of pemphigus vulgarisCutis200473640340615224785
   PubMed Web of Science ®Google Scholar
• VenturaFRochaJFernandesJCMachadoABritoCRecalcitrant pemphigus vulgaris: aseptic meningitis associated with intravenous immunoglobulin therapy and successful treatment with rituximabInt J Dermatol201352450150223451774
   PubMed Web of Science ®Google Scholar
• OrbachHKatzUShererYShoenfeldYIntravenous immunoglobulin: adverse effects and safe administrationClin Rev Allergy Immunol200529317318416391392
   PubMed Web of Science ®Google Scholar
• KasperkiewiczMZillikensDThe pathophysiology of bullous pemphigoidClin Rev Allergy Immunol2007331–2677718094948
   PubMed Web of Science ®Google Scholar
• TurnerMSSuttonDSauderDNThe use of plasmapheresis and immunosuppression in the treatment of pemphigus vulgarisJ Am Acad Dermatol20004361058106411100023
   PubMed Web of Science ®Google Scholar
• RoujeauJCAndreCJoneau FabreMPlasma exchange in pemphigus: uncontrolled study of ten patientsArch Dermatol198311932152216824359
   PubMedGoogle Scholar
• Tan-LimRBystrynJCEffect of plasmapheresis therapy on circulating levels of pemphigus antibodiesJ Am Acad Dermatol199022135402298963
   PubMed Web of Science ®Google Scholar
• RanughaPSKumariRKarthaLBParameswaranSThappaDMTherapeutic plasma exchange as a crisis option in severe pemphigus vulgarisIndian J Dermatol Venereol Leprol201278450851022772632
   PubMed Web of Science ®Google Scholar
• KasuyaAMorikiMTatsunoKHirakawaSTokuraYClearance efficacy of autoantibodies in double filtration plasmapheresis for pemphigus foliaceusActa Derm Venereol201393218118222948906
   PubMed Web of Science ®Google Scholar
• YehSWSamiNAhmedRATreatment of pemphigus vulgaris: current and emerging optionsAm J Clin Dermatol20056532734216252932
   PubMed Web of Science ®Google Scholar
• SagiLBaumSGendelmanVTrauHBarzilaiAThe role of therapeutic plasma exchange in pemphigus vulgarisJ Eur Acad Dermatol Venereol2011251828620477926
   PubMed Web of Science ®Google Scholar
• GuillaumeJCRoujeauJCMorelPControlled study of plasma exchange in pemphigusArch Dermatol198812411165916633178248
   PubMedGoogle Scholar
• BraunNKadarJGRislerTTherapeutic immunoadsorption – its role in clinical practiceTransfus Sci199819Suppl656910178698
   PubMedGoogle Scholar
• MeyersburgDSchmidtEKasperkiewiczMZillikensDImmunoad-sorption in dermatologyTher Apher Dial201216431132022817118
   PubMed Web of Science ®Google Scholar
• KasperkiewiczMShimanovichIMeierMTreatment of severe pemphigus with a combination of immunoadsorption, rituximab, pulsed dexamethasone and azathioprine/mycophenolate mofetil: a pilot study of 23 patientsBr J Dermatol2012166115416021910700
   PubMed Web of Science ®Google Scholar
• ZillikensDDerflerKEmingRRecommendations for the use of immunoapheresis in the treatment of autoimmune bullous diseasesJ Dtsch Dermatol Ges200751088188717910670
   PubMedGoogle Scholar
• LüftlMStauberAMainkaAKlingelRSchulerGHertlMSuccessful removal of pathogenic autoantibodies in pemphigus by immunoadsorption with a tryptophan-linked polyvinylalcohol adsorberBr J Dermatol2003149359860514510995
   PubMed Web of Science ®Google Scholar
• FrostNMesserGFierlbeckGRislerTLyttonSDTreatment of pemphigus vulgaris with protein A immunoadsorption: case report of long-term history showing favorable outcomeAnn N Y Acad Sci2005105159159616126999
   PubMed Web of Science ®Google Scholar
• EmingRHertlMImmunoadsorption in pemphigusAutoimmunity200639760961617101505
   PubMed Web of Science ®Google Scholar
• SchneiderKMPlasmapheresis and immunoadsorption: different techniques and their current role in medical therapyKidney Int Suppl199864S61S659475492
   PubMed Web of Science ®Google Scholar
• SchmidtEZillikensDImmunoadsorption in dermatologyArch Dermatol Res2010302424125320049466
   PubMed Web of Science ®Google Scholar
• ZhaoCYMurrellDFPemphigus vulgaris: an evidence-based treatment updateDrugs201575327128425655250
   PubMed Web of Science ®Google Scholar
• WerthVPFivensonDPandyaAGMulticenter randomized, double-blind, placebo-controlled, clinical trial of dapsone as a glucocorticoid-sparing agent in maintenance-phase pemphigus vulgarisArch Dermatol20081441253218209165
   PubMedGoogle Scholar
• GürcanHMAhmedAREfficacy of dapsone in the treatment of pemphigus and pemphigoid: analysis of current dataAm J Clin Dermatol200910638339619824739
   PubMed Web of Science ®Google Scholar
• CiprianiPRuscittiPCarubbiFLiakouliVGiacomelliRMethotrexate: an old new drug in autoimmune diseaseExpert Rev Clin Immunol201410111519153025245537
   PubMed Web of Science ®Google Scholar
• BaumSGreenbergerSSamuelovLMethotrexate is an effective and safe adjuvant therapy for pemphigus vulgarisEur J Dermatol2012221838722266247
   PubMed Web of Science ®Google Scholar
• TranKDWolvertonJESoterNAMethotrexate in the treatment of pemphigus vulgaris: experience in 23 patientsBr J Dermatol2013169491692123772610
   PubMed Web of Science ®Google Scholar
• MatsudaSKoyasuSMechanisms of action of cyclosporineImmunopharmacology2000472–311912510878286
   PubMed Web of Science ®Google Scholar
• DollerAAkoolel-SMüllerRMolecular mechanisms of cyclosporin A inhibition of the cytokine-induced matrix metalloproteinase-9 in glomerular mesangial cellsJ Am Soc Nephrol200718258159217202418
   PubMed Web of Science ®Google Scholar
• IoannidesDChrysomallisFBystrynJCIneffectiveness of cyclosporine as an adjuvant to corticosteroids in the treatment of pemphigusArch Dermatol2000136786887210890988
   PubMedGoogle Scholar
• EllebrechtCTBhojVGNaceAReengineering chimeric antigen receptor T cells for targeted therapy of autoimmune diseaseScience2016353629517918427365313
   PubMed Web of Science ®Google Scholar
• HertlMAmagaiMSundaramHStanleyJIshiiKKatzSIRecognition of desmoglein 3 by autoreactive T cells in pemphigus vulgaris patients and normalsJ Invest Dermatol1998110162669424089
   PubMed Web of Science ®Google Scholar
• ChowSRizzoCRavitskiyLSinhaAAThe role of T cells in cutaneous autoimmune diseaseAutoimmunity200538430331716206513
   PubMed Web of Science ®Google Scholar
• Aoki-OtaMKinoshitaMOtaTTolerance induction by the blockade of CD40/CD154 interaction in pemphigus vulgaris mouse modelJ Invest Dermatol2006126110511316417225
   PubMed Web of Science ®Google Scholar
• TakahashiHAmagaiMNishikawaTFujiiYKawakamiYKuwanaMNovel system evaluating in vivo pathogenicity of desmoglein 3-reactive T cell clones using murine pemphigus ulgarisJ Immunol200818121526153518606708
   PubMed Web of Science ®Google Scholar
• AnhaltGWerthVStroberBAn open-label phase I clinical study to assess the safety of PI-0824 in patients with pemphigus vulgarisJ Invest Dermatol200512551088
   Web of Science ®Google Scholar
• SchneiderPMacKayFSteinerVBAFF, a novel ligand of the tumor necrosis factor family, stimulates B cell growthJ Exp Med1999189111747175610359578
   PubMed Web of Science ®Google Scholar
• HahneMKataokaTSchröterMAPRIL, a new ligand of the tumor necrosis factor family, stimulates tumor cell growthJ Exp Med19981886118511909743536
   PubMed Web of Science ®Google Scholar
• LitinskiyMBNardelliBHilbertDMDCs induce CD40-independent immunoglobulin class switching through BLyS and APRILNat Immunol20023982282912154359
   PubMed Web of Science ®Google Scholar
• BossenCSchneiderPBAFF, APRIL and their receptors: structure, function and signalingSemin Immunol200618526327516914324
   PubMed Web of Science ®Google Scholar
• TangyeSGBryantVLCussAKGoodKLBAFF, APRIL and human B cell disordersSemin Immunol200618530531716916610
   PubMed Web of Science ®Google Scholar
• AsashimaNFujimotoMWatanabeRSerum levels of BAFF are increased in bullous pemphigoid but not in pemphigus vulgarisBr J Dermatol2006155233033616882171
   PubMed Web of Science ®Google Scholar
• NagelAPodstawaEEickmannMMüllerHHHertlMEmingRRituximab mediates a strong elevation of B-cell-activating factor associated with increased pathogen-specific IgG but not autoantibodies in pemphigus vulgarisJ Invest Dermatol200912992202221019282839
   PubMed Web of Science ®Google Scholar
• SinhaAAHoffmanMBJanickeECPemphigus vulgaris: approach to treatmentEur J Dermatol201525210311325547117
   PubMed Web of Science ®Google Scholar
• BerkowitzPHuPLiuZDesmosome signaling. Inhibition of p38MAPK prevents pemphigus vulgaris IgG-induced cytoskeleton reorganizationJ Biol Chem200528025237782378415840580
   PubMed Web of Science ®Google Scholar
• BerkowitzPHuPWarrenSLiuZDiazLARubensteinDSp38MAPK inhibition prevents disease in pemphigus vulgaris miceProc Natl Acad Sci U S A200610334128551286016908851
   PubMed Web of Science ®Google Scholar
• YoshidaKIshiiKShimizuANon-pathogenic pemphigus foliaceus (PF) IgG acts synergistically with a directly pathogenic PF IgG to increase blistering by p38MAPK-dependent desmoglein 1 clusteringJ Dermatol Sci201785319720728024684
   PubMed Web of Science ®Google Scholar
• MaoXSanoYParkJMPayneASp38 MAPK activation is downstream of the loss of intercellular adhesion in pemphigus vulgarisJ Biol Chem201128621283129121078676
   PubMed Web of Science ®Google Scholar
• SweeneySEFiresteinGSMitogen activated protein kinase inhibitors: where are we now and where are we going?Ann Rheum Dis200665Suppl 3iii83iii8817038480
   PubMedGoogle Scholar
• SpindlerVVielmuthFSchmidtERubensteinDSWaschkeJProtective endogenous cyclic adenosine 5′-monophosphate signaling triggered by pemphigus autoantibodiesJ Immunol2010185116831683821037102
   PubMed Web of Science ®Google Scholar
• GrandoSAPittelkowMRSchallreuterKUAdrenergic and cholinergic control in the biology of epidermis: Physiological and clinical significanceJ Invest Dermatol200612691948196516912692
   PubMed Web of Science ®Google Scholar
• WilliamsonLRaessNACaldelariRPemphigus vulgaris identifies plakoglobin as key suppressor of c-Myc in the skinEMBO J200625143298330916871158
   PubMed Web of Science ®Google Scholar
• BektasMJollyPSBerkowitzPAmagaiMRubensteinDSA pathophysiologic role for epidermal growth factor receptor in pemphigus acantholysisJ Biol Chem2013288139447945623404504
   PubMed Web of Science ®Google Scholar
• OuterbridgeCDavisDWhiteSA new treatment for autoimmune blistering diseases – the efficacy of the Bruton’s tyrosine kinase inhibitor PRN473 in canine pemphigus foliaceusJ Am Acad Dermatol2016745AB141
   Web of Science ®Google Scholar
• SmithCEBerglöfAX-linked agammaglobulinemiaAdamMPArdingerHHPagonRAWallaceSEBeanLJHStephensKAmemiyaASourceGeneReviews® [Internet]Seattle, WAUniversity of Washington19932018 Available from: http://www.ncbi.nlm.nih.gov/pubmed/20301626Accessed February 23, 2018
   Google Scholar
• Principia Biopharma, IncA study of PRN1008 in adult patients with pemphigus vulgaris Available from: https://clinicaltrials.gov/ct2/show/NCT02704429?cond=pemphigus&rank=10. NLM identifier: NCT02704429Accessed February 23, 2018
   Google Scholar
• IqbalMMOsteoporosis: epidemiology, diagnosis, and treatmentSouth Med J200093121810653058
   PubMed Web of Science ®Google Scholar
• GrossmanJMGordonRRanganathVKAmerican College of Rheumatology 2010 recommendations for the prevention and treatment of glucocorticoid-induced osteoporosisArthritis Care Res (Hoboken)201062111515152620662044
   PubMed Web of Science ®Google Scholar
• SchadtCJacksonSGlucocorticoidsBologniaJLSchafferJVJorizzoJLDermatology4th edPhiladelphiaElsevier201721862199
   Google Scholar
• DesforgesJFMankinHJNontraumatic necrosis of bone (osteonecrosis)N Engl J Med199232622147314791574093
   PubMed Web of Science ®Google Scholar
• GuptaYGuptaAGlucocorticoid-induced myopathy: pathophysiology, diagnosis, and treatmentIndian J Endocrinol Metab201317591391624083177
   PubMedGoogle Scholar
• AllenDBGrowth suppression by glucocorticoid therapyEndocrinol Metab Clin North Am19962536997178879994
   PubMed Web of Science ®Google Scholar
• RenfroLSnowJSOcular effects of topical and systemic steroidsDermatol Clin19921035055121617809
   PubMed Web of Science ®Google Scholar
• WhitworthJAMechanisms of glucocorticoid-induced hypertensionKidney Int1987315121312243298796
   PubMed Web of Science ®Google Scholar
• PiperJMRayWADaughertyJRGriffinMRCorticosteroid use and peptic ulcer disease: role of nonsteroidal anti-inflammatory drugsAnn Intern Med199111497357402012355
   PubMed Web of Science ®Google Scholar
• AmberKTLambertsASolimaniFDetermining the incidence of Pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxisJAMA Dermatol2017153111137114128854309
   PubMed Web of Science ®Google Scholar
• FardetLFlahaultAKettanehACorticosteroid-induced clinical adverse events: frequency, risk factors and patient’s opinionBr J Dermatol2007157114214817501951
   PubMed Web of Science ®Google Scholar
• TabriziMNChams-DavatchiCEsmaeeliNAccelerating effects of epidermal growth factor on skin lesions of pemphigus vulgaris: a double-blind, randomized, controlled trialJ Eur Acad Dermatol Venereol2007211798417207172
   PubMed Web of Science ®Google Scholar
• el-DaroutiMMarzoukSAbdel HayRThe use of sulfasalazine and pentoxifylline (low-cost antitumour necrosis factor drugs) as adjuvant therapy for the treatment of pemphigus vulgaris: a comparative studyBr J Dermatol2009161231331919466963
   PubMed Web of Science ®Google Scholar
• IrajiFAsilianASiadatAHPimecrolimus 1% cream in the treatment of cutaneous lesions of pemphigus vulgaris: a double-blind, placebo-controlled clinical trialJ Drugs Dermatol20109668468620645531
   PubMed Web of Science ®Google Scholar
• FiorentinoDFGarciaMSRehmusWKimballABA pilot study of etanercept treatment for pemphigus vulgarisArch Dermatol2011147111711821242406
   PubMedGoogle Scholar
• Nazemi-TabriziMJHatamiPGhiasiMDaneshpazhoohMChams-DavatchiCRandomized trial of tacrolimus 0.1% ointment versus triamcinolone acetonide 0.1% paste in the treatment of oral pemphigus vulgarisIran J Dermatol201215604246
   Google Scholar
• HallRP3rdFairleyJWoodleyDA multicentre randomized trial of the treatment of patients with pemphigus vulgaris with infliximab and prednisone compared with prednisone aloneBr J Dermatol2015172376076825123295
   PubMed Web of Science ®Google Scholar
• IrajiFFaghihiGSiadatAHThe efficacy of acyclovir in treatment of the pemphigus vulgarisJ Res Med Sci2013181197697824523784
   PubMed Web of Science ®Google Scholar
• KanwarAJVinayKSawatkarGUClinical and immunological outcomes of high- and low-dose rituximab treatments in patients with pemphigus: a randomized, comparative, observer-blinded studyBr J Dermatol201417061341134924640990
   PubMed Web of Science ®Google Scholar
• DastgheibLSadatiMSBaghernejhadMAssessment of the adjuvant effect of tacrolimus in the management of pemphigus vulgaris: a randomized controlled trialJ Dermatolog Treat2015261909324552352
   PubMed Web of Science ®Google Scholar
• El-DaroutiMAHegazyRAAbdel HayRMStudy of T helper 1 and T helper 2 responses in pemphigus vulgaris patients receiving interferon alpha 2a injections in addition to a standard protocol therapy: a randomized controlled trialArch Dermatol Res2015307429930725450635
   PubMed Web of Science ®Google Scholar