No.	Month	Year	Category	Country	Citation	Journal	Type	PMID	Article
1	84	2016	immunology	U.S	123	Science	Journal Article	27365313	Ellebrecht CT, Bhoj VG, Nace A, Choi EJ, Mao X, Cho MJ, Di Zenzo G, Lanzavecchia A, Seykora JT, Cotsarelis G, Milone MC, Payne AS. Reengineering chimeric antigen receptor T cells for targeted therapy of autoimmune disease. Science. 2016 Jul 8;353(6295):179-84. doi: 10.1126/science.aaf6756. Epub 2016 Jun 30
2	63	2017	administration & dosage	France	77	Lancet	Comparative Study	28342637	Joly P, Maho-Vaillant M, Prost-Squarcioni C, Hebert V, Houivet E, Calbo S, Caillot F, Golinski ML, Labeille B, Picard-Dahan C, Paul C, Richard MA, Bouaziz JD, Duvert-Lehembre S, Bernard P, Caux F, Alexandre M, Ingen-Housz-Oro S, Vabres P, Delaporte E, Quereux G, Dupuy A, Debarbieux S, Avenel-Audran M, D'Incan M, Bedane C, Bénéton N, Jullien D, Dupin N, Misery L, Machet L, Beylot-Barry M, Dereure O, Sassolas B, Vermeulin T, Benichou J, Musette P. 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 May 20;389(10083):2031-2040. doi: 10.1016/S0140-6736(17)30070-3. Epub 2017 Mar 22.
3	111	2017	diagnosis	Germany	64	Nat Rev Dis Primers	Journal Article	28492232	Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS, Amagai M. Pemphigus. Nat Rev Dis Primers. 2017 May 11;3:17026. doi: 10.1038/nrdp.2017.26.
4	111	2012	physiology	Japan	63	J Invest Dermatol	Journal Article	22189787	Amagai M, Stanley JR. Desmoglein as a target in skin disease and beyond. J Invest Dermatol. 2012 Mar;132(3 Pt 2):776-84. doi: 10.1038/jid.2011.390. Epub 2011 Dec 22.
5	193	2011	administration & dosage	Germany	60	Arthritis Res Ther	Journal Article	21569519	Tony HP, Burmester G, Schulze-Koops H, Grunke M, Henes J, Kötter I, Haas J, Unger L, Lovric S, Haubitz M, Fischer-Betz R, Chehab G, Rubbert-Roth A, Specker C, Weinerth J, Holle J, Müller-Ladner U, König R, Fiehn C, Burgwinkel P, Budde K, Sörensen H, Meurer M, Aringer M, Kieseier B, Erfurt-Berge C, Sticherling M, Veelken R, Ziemann U, Strutz F, von Wussow P, Meier FM, Hunzelmann N, Schmidt E, Bergner R, Schwarting A, Eming R, Hertl M, Stadler R, Schwarz-Eywill M, Wassenberg S, Fleck M, Metzler C, Zettl U, Westphal J, Heitmann S, Herzog AL, Wiendl H, Jakob W, Schmidt E, Freivogel K, Dörner T. Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID). Arthritis Res Ther. 2011 May 13;13(3):R75. doi: 10.1186/ar3337.
6	111	2013	therapeutic use	France	56	Sci Transl Med	Journal Article	23467561	Colliou N, Picard D, Caillot F, Calbo S, Le Corre S, Lim A, Lemercier B, Le Mauff B, Maho-Vaillant M, Jacquot S, Bedane C, Bernard P, Caux F, Prost C, Delaporte E, Doutre MS, Dreno B, Franck N, Ingen-Housz-Oro S, Chosidow O, Pauwels C, Picard C, Roujeau JC, Sigal M, Tancrede-Bohin E, Templier I, Eming R, Hertl M, D'Incan M, Joly P, Musette P. Long-term remissions of severe pemphigus after rituximab therapy are associated with prolonged failure of desmoglein B cell response. Sci Transl Med. 2013 Mar 6;5(175):175ra30. doi: 10.1126/scitranslmed.3005166.
7	84	2012	immunology	Italy	55	J Clin Invest	Journal Article	22996451	Di Zenzo G, Di Lullo G, Corti D, Calabresi V, Sinistro A, Vanzetta F, Didona B, Cianchini G, Hertl M, Eming R, Amagai M, Ohyama B, Hashimoto T, Sloostra J, Sallusto F, Zambruno G, Lanzavecchia A. Pemphigus autoantibodies generated through somatic mutations target the desmoglein-3 cis-interface. J Clin Invest. 2012 Oct;122(10):3781-90. doi: 10.1172/JCI64413. Epub 2012 Sep 4.
8	111	2016	immunology	U.S	49	Annu Rev Pathol	Journal Article	26907530	Hammers CM, Stanley JR. Mechanisms of Disease: Pemphigus and Bullous Pemphigoid. Annu Rev Pathol. 2016 May 23;11:175-97. doi: 10.1146/annurev-pathol-012615-044313. Epub 2016 Feb 22.
9	111	2012	immunology	U.S	48	Autoimmunity	Journal Article	21939410	Grando SA. Pemphigus autoimmunity: hypotheses and realities. Autoimmunity. 2012 Feb;45(1):7-35. doi: 10.3109/08916934.2011.606444. Epub 2011 Sep 23
10	111	2012	genetics	China	40	Genes Immun	Journal Article	23076337	Zheng J, Ibrahim S, Petersen F, Yu X. Meta-analysis reveals an association of PTPN22 C1858T with autoimmune diseases, which depends on the localization of the affected tissue. Genes Immun. 2012 Dec;13(8):641-52. doi: 10.1038/gene.2012.46. Epub 2012 Oct 18.
11	191	2015	diagnosis	Germany	39	J Eur Acad Dermatol Venereol	Journal Article	25338479	Hertl M, Jedlickova H, Karpati S, Marinovic B, Uzun S, Yayli S, Mimouni D, Borradori L, Feliciani C, Ioannides D, Joly P, Kowalewski C, Zambruno G, Zillikens D, Jonkman MF. Pemphigus. 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 Venereol. 2015 Mar;29(3):405-14. doi: 10.1111/jdv.12772. Epub 2014 Oct 22.
12	111	2011	physiology	U.S	39	J Biol Chem	Journal Article	21078676	Mao X, Sano Y, Park JM, Payne AS. p38 MAPK activation is downstream of the loss of intercellular adhesion in pemphigus vulgaris. J Biol Chem. 2011 Jan 14;286(2):1283-91. doi: 10.1074/jbc.M110.172874. Epub 2010 Nov 15
13	475	2013	chemistry	Germany	38	J Clin Invest	Journal Article	23298835	Spindler V, Rötzer V, Dehner C, Kempf B, Gliem M, Radeva M, Hartlieb E, Harms GS, Schmidt E, Waschke J. Peptide-mediated desmoglein 3 crosslinking prevents pemphigus vulgaris autoantibody-induced skin blistering. J Clin Invest. 2013 Feb;123(2):800-11. doi: 10.1172/JCI60139. Epub 2013 Jan 9.
14	130	2012	complications	U.S	38	PLoS One	Journal Article	23226536	Saito M, Stahley SN, Caughman CY, Mao X, Tucker DK, Payne AS, Amagai M, Kowalczyk AP. Signaling dependent and independent mechanisms in pemphigus vulgaris blister formation. PLoS One. 2012;7(12):e50696. doi: 10.1371/journal.pone.0050696. Epub 2012 Dec 3.
15	99	2016	age factors	Germany	33	J Invest Dermatol	Letter	27456755	Hübner F, Recke A, Zillikens D, Linder R, Schmidt E. Prevalence and Age Distribution of Pemphigus and Pemphigoid Diseases in Germany. J Invest Dermatol. 2016 Dec;136(12):2495-2498. doi: 10.1016/j.jid.2016.07.013. Epub 2016 Jul 25.
16	111	2011	diagnosis	Germany	33	J Dtsch Dermatol Ges	Journal Article	21955378	Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 1: Clinical manifestations. J Dtsch Dermatol Ges. 2011 Oct;9(10):844-56; quiz 857. doi: 10.1111/j.1610-0387.2011.07793.x.
17	111	2011	pharmacology	U.S	33	J Invest Dermatol	Journal Article	21160493	Jennings JM, Tucker DK, Kottke MD, Saito M, Delva E, Hanakawa Y, Amagai M, Kowalczyk AP. Desmosome disassembly in response to pemphigus vulgaris IgG occurs in distinct phases and can be reversed by expression of exogenous Dsg3. J Invest Dermatol. 2011 Mar;131(3):706-18. doi: 10.1038/jid.2010.389. Epub 2010 Dec 16
18	85	2013	genetics	U.K	32	J Mol Biol	Journal Article	23911551	Al-Jassar C, Bikker H, Overduin M, Chidgey M. Mechanistic basis of desmosome-targeted diseases. J Mol Biol. 2013 Nov 1;425(21):4006-22. doi: 10.1016/j.jmb.2013.07.035. Epub 2013 Aug 2.
19	193	2014	metabolism	U.S	30	PLoS One	Journal Article	24498201	Stahley SN, Saito M, Faundez V, Koval M, Mattheyses AL, Kowalczyk AP. Desmosome assembly and disassembly are membrane raft-dependent. PLoS One. 2014 Jan 30;9(1):e87809. doi: 10.1371/journal.pone.0087809. eCollection 2014
20	154	2011	therapeutic use	Germany	30	J Am Acad Dermatol	Journal Article	21641080	Kasperkiewicz M, Shimanovich I, Ludwig RJ, Rose C, Zillikens D, Schmidt E. Rituximab for treatment-refractory pemphigus and pemphigoid: a case series of 17 patients. J Am Acad Dermatol. 2011 Sep;65(3):552-558. doi: 10.1016/j.jaad.2010.07.032.
21	317	2020	administration & dosage	Australia	28	J Am Acad Dermatol	Journal Article	29438767	Murrell DF, Peña S, Joly P, Marinovic B, Hashimoto T, Diaz LA, Sinha AA, Payne AS, Daneshpazhooh M, Eming R, Jonkman MF, Mimouni D, Borradori L, Kim SC, Yamagami J, Lehman JS, Saleh MA, Culton DA, Czernik A, Zone JJ, Fivenson D, Ujiie H, Wozniak K, Akman-Karaka? A, Bernard P, Korman NJ, Caux F, Drenovska K, Prost-Squarcioni C, Vassileva S, Feldman RJ, Cardones AR, Bauer J, Ioannides D, Jedlickova H, Palisson F, Patsatsi A, Uzun S, Yayli S, Zillikens D, Amagai M, Hertl M, Schmidt E, Aoki V, Grando SA, Shimizu H, Baum S, Cianchini G, Feliciani C, Iranzo P, Mascaró JM Jr, Kowalewski C, Hall R, Groves R, Harman KE, Marinkovich MP, Maverakis E, Werth VP. Diagnosis and management of pemphigus: Recommendations of an international panel of experts. J Am Acad Dermatol. 2020 Mar;82(3):575-585.e1. doi: 10.1016/j.jaad.2018.02.021. Epub 2018 Feb 10.
22	89	2013	metabolism	Germany	28	PLoS One	Journal Article	23326495	Hartlieb E, Kempf B, Partilla M, Vigh B, Spindler V, Waschke J. Desmoglein 2 is less important than desmoglein 3 for keratinocyte cohesion. PLoS One. 2013;8(1):e53739. doi: 10.1371/journal.pone.0053739. Epub 2013 Jan 11.
23	134	2015	epidemiology	Turkey	27	Arch Dermatol Res	Journal Article	25589418	Alpsoy E, Akman-Karakas A, Uzun S. Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid. Arch Dermatol Res. 2015 May;307(4):291-8. doi: 10.1007/s00403-014-1531-1. Epub 2015 Jan 15.
24	19	2013	blood	U.S	27	PLoS One	Journal Article	23505434	Kalantari-Dehaghi M, Anhalt GJ, Camilleri MJ, Chernyavsky AI, Chun S, Felgner PL, Jasinskas A, Leiferman KM, Liang L, Marchenko S, Nakajima-Sasaki R, Pittelkow MR, Zone JJ, Grando SA. Pemphigus vulgaris autoantibody profiling by proteomic technique. PLoS One. 2013;8(3):e57587. doi: 10.1371/journal.pone.0057587. Epub 2013 Mar 7.
25	148	2012	diagnosis	Germany	27	Orphanet J Rare Dis	Comparative Study	22876746	van Beek N, Rentzsch K, Probst C, Komorowski L, Kasperkiewicz M, Fechner K, Bloecker IM, Zillikens D, Stöcker W, Schmidt E. Serological diagnosis of autoimmune bullous skin diseases: prospective comparison of the BIOCHIP mosaic-based indirect immunofluorescence technique with the conventional multi-step single test strategy. Orphanet J Rare Dis. 2012 Aug 9;7:49. doi: 10.1186/1750-1172-7-49.
26	111	2011	metabolism	Netherlands	27	Br J Dermatol	Journal Article	21692763	Oktarina DA, van der Wier G, Diercks GF, Jonkman MF, Pas HH. IgG-induced clustering of desmogleins 1 and 3 in skin of patients with pemphigus fits with the desmoglein nonassembly depletion hypothesis. Br J Dermatol. 2011 Sep;165(3):552-62. doi: 10.1111/j.1365-2133.2011.10463.x.
27	129	2018	immunology	Germany	26	J Invest Dermatol	Journal Article	29037765	Spindler V, Eming R, Schmidt E, Amagai M, Grando S, Jonkman MF, Kowalczyk AP, Müller EJ, Payne AS, Pincelli C, Sinha AA, Sprecher E, Zillikens D, Hertl M, Waschke J. Mechanisms Causing Loss of Keratinocyte Cohesion in Pemphigus. J Invest Dermatol. 2018 Jan;138(1):32-37. doi: 10.1016/j.jid.2017.06.022. Epub 2017 Oct 14.
28	62	2015	pathology	U.S	26	J Invest Dermatol	Case Reports	25142730	Hammers CM, Chen J, Lin C, Kacir S, Siegel DL, Payne AS, Stanley JR. Persistence of anti-desmoglein 3 IgG(+) B-cell clones in pemphigus patients over years. J Invest Dermatol. 2015 Mar;135(3):742-749. doi: 10.1038/jid.2014.291. Epub 2014 Jul 14
29	111	2012	genetics	Israel	26	J Invest Dermatol	Journal Article	22437316	Sarig O, Bercovici S, Zoller L, Goldberg I, Indelman M, Nahum S, Israeli S, Sagiv N, Martinez de Morentin H, Katz O, Baum S, Barzilai A, Trau H, Murrell DF, Bergman R, Hertl M, Rosenberg S, Nöthen MM, Skorecki K, Schmidt E, Zillikens D, Darvasi A, Geiger D, Rosset S, Ibrahim SM, Sprecher E. Population-specific association between a polymorphic variant in ST18, encoding a pro-apoptotic molecule, and pemphigus vulgaris. J Invest Dermatol. 2012 Jul;132(7):1798-805. doi: 10.1038/jid.2012.46. Epub 2012 Mar 22
30	246	2011	diagnosis		26	Dtsch Arztebl Int	Journal Article	21712975	Schmidt E, Zillikens D. The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Arztebl Int. 2011 Jun;108(23):399-405, I-III. doi: 10.3238/arztebl.2011.0399. Epub 2011 Jun 10.
31	111	2018	immunology	Israel	25	Immunol Res	Journal Article	29479654	Kridin K. Pemphigus group: overview, epidemiology, mortality, and comorbidities. Immunol Res. 2018 Apr;66(2):255-270. doi: 10.1007/s12026-018-8986-7.
32	111	2015	standards	Germany	25	J Dtsch Dermatol Ges	Journal Article	26110729	Schmidt E, Goebeler M, Hertl M, Sárdy M, Sitaru C, Eming R, Hofmann SC, Hunzelmann N, Kern JS, Kramer H, Orzechowski HD, Pfeiffer C, Schuster V, Sporbeck B, Sticherling M, Worm M, Zillikens D, Nast A. S2k guideline for the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges. 2015 Jul;13(7):713-27. doi: 10.1111/ddg.12612.
33	117	2012	genetics	Brazil	25	PLoS One	Clinical Trial	22768326	Augusto DG, Lobo-Alves SC, Melo MF, Pereira NF, Petzl-Erler ML. Activating KIR and HLA Bw4 ligands are associated to decreased susceptibility to pemphigus foliaceus, an autoimmune blistering skin disease. PLoS One. 2012;7(7):e39991. doi: 10.1371/journal.pone.0039991. Epub 2012 Jul 2.
34	111	2011	diagnosis	Germany	25	J Dtsch Dermatol Ges	Journal Article	22026362	Kneisel A, Hertl M. Autoimmune bullous skin diseases. Part 2: diagnosis and therapy. J Dtsch Dermatol Ges. 2011 Nov;9(11):927-47. doi: 10.1111/j.1610-0387.2011.07809.x.
35	67	2011	therapeutic use	Australia	25	J Am Acad Dermatol	Journal Article	21353333	Martin LK, Werth VP, Villaneuva EV, Murrell DF. A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol. 2011 May;64(5):903-8. doi: 10.1016/j.jaad.2010.04.039. Epub 2011 Feb 25.
36	111	2018	immunology	Germany	24	Clin Rev Allergy Immunol	Journal Article	29313220	Pollmann R, Schmidt T, Eming R, Hertl M. Pemphigus: a Comprehensive Review on Pathogenesis, Clinical Presentation and Novel Therapeutic Approaches. Clin Rev Allergy Immunol. 2018 Feb;54(1):1-25. doi: 10.1007/s12016-017-8662-z.
37	46	2015	ultrastructure	Netherlands	24	J Invest Dermatol	Journal Article	25789704	Sokol E, Kramer D, Diercks GFH, Kuipers J, Jonkman MF, Pas HH, Giepmans BNG. Large-Scale Electron Microscopy Maps of Patient Skin and Mucosa Provide Insight into Pathogenesis of Blistering Diseases. J Invest Dermatol. 2015 Jul;135(7):1763-1770. doi: 10.1038/jid.2015.109. Epub 2015 Mar 19.
38	53	2014	genetics	U.S	24	Nat Commun	Journal Article	24942562	Cho MJ, Lo AS, Mao X, Nagler AR, Ellebrecht CT, Mukherjee EM, Hammers CM, Choi EJ, Sharma PM, Uduman M, Li H, Rux AH, Farber SA, Rubin CB, Kleinstein SH, Sachais BS, Posner MR, Cavacini LA, Payne AS. Shared VH1-46 gene usage by pemphigus vulgaris autoantibodies indicates common humoral immune responses among patients. Nat Commun. 2014 Jun 19;5:4167. doi: 10.1038/ncomms5167.
39	37	2014	diagnosis	Israel	24	Autoimmun Rev	Journal Article	24434358	Baum S, Sakka N, Artsi O, Trau H, Barzilai A. Diagnosis and classification of autoimmune blistering diseases. Autoimmun Rev. 2014 Apr-May;13(4-5):482-9. doi: 10.1016/j.autrev.2014.01.047. Epub 2014 Jan 13.
40	111	2012	therapeutic use	U.S	24	Arch Dermatol	Journal Article	22710375	Lunardon L, Tsai KJ, Propert KJ, Fett N, Stanley JR, Werth VP, Tsai DE, Payne AS. Adjuvant rituximab therapy of pemphigus: a single-center experience with 31 patients. Arch Dermatol. 2012 Sep;148(9):1031-6. doi: 10.1001/archdermatol.2012.1522.
41	111	2015	administration & dosage	Taiwan	23	Acta Derm Venereol	Journal Article	25881672	Wang HH, Liu CW, Li YC, Huang YC. Efficacy of rituximab for pemphigus: a systematic review and meta-analysis of different regimens. Acta Derm Venereol. 2015 Nov;95(8):928-32. doi: 10.2340/00015555-2116.
42	591	2014	diagnosis	Israel	23	Autoimmun Rev	Journal Article	24424192	Kershenovich R, Hodak E, Mimouni D. Diagnosis and classification of pemphigus and bullous pemphigoid. Autoimmun Rev. 2014 Apr-May;13(4-5):477-81. doi: 10.1016/j.autrev.2014.01.011. Epub 2014 Jan 11.
43	111	2013	immunology	Italy	23	Clin Dermatol	Journal Article	23806154	Ruocco V, Ruocco E, Lo Schiavo A, Brunetti G, Guerrera LP, Wolf R. Pemphigus: etiology, pathogenesis, and inducing or triggering factors: facts and controversies. Clin Dermatol. 2013 Jul-Aug;31(4):374-381. doi: 10.1016/j.clindermatol.2013.01.004.
44	48	2018	immunology	Switzerland	22	Front Immunol	Journal Article	29449846	Spindler V, Waschke J. Pemphigus-A Disease of Desmosome Dysfunction Caused by Multiple Mechanisms. Front Immunol. 2018 Feb 1;9:136. doi: 10.3389/fimmu.2018.00136. eCollection 2018.
45	111	2015	administration & dosage	Germany	22	J Dtsch Dermatol Ges	Journal Article	26213827	Eming R, Sticherling M, Hofmann SC, Hunzelmann N, Kern JS, Kramer H, Pfeiffer C, Schuster V, Zillikens D, Goebeler M, Hertl M, Nast A, Orzechowski HD, Sárdy M, Schmidt E, Sitaru C, Sporbeck B, Worm M. S2k guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges. 2015 Aug;13(8):833-44. doi: 10.1111/ddg.12606.
46	111	2013	metabolism	U.S	22	J Biol Chem	Journal Article	23404504	Bektas M, Jolly PS, Berkowitz P, Amagai M, Rubenstein DS. A pathophysiologic role for epidermal growth factor receptor in pemphigus acantholysis. J Biol Chem. 2013 Mar 29;288(13):9447-56. doi: 10.1074/jbc.M112.438010. Epub 2013 Feb 12.
47	111	2014	metabolism	Germany	21	Med Res Rev	Journal Article	24549583	Waschke J, Spindler V. Desmosomes and extradesmosomal adhesive signaling contacts in pemphigus. Med Res Rev. 2014 Nov;34(6):1127-45. doi: 10.1002/med.21310. Epub 2014 Feb 18.
48	7	2011	blood	Israel	21	Autoimmun Rev	Journal Article	21527361	Sagi L, Baum S, Agmon-Levin N, Sherer Y, Katz BS, Barzilai O, Ram M, Bizzaro N, SanMarco M, Trau H, Shoenfeld Y. Autoimmune bullous diseases the spectrum of infectious agent antibodies and review of the literature. Autoimmun Rev. 2011 Jul;10(9):527-35. doi: 10.1016/j.autrev.2011.04.003. Epub 2011 Apr 16.
49	99	2017	metabolism	Germany	20	Sci Rep	Journal Article	28620161	Walter E, Vielmuth F, Rotkopf L, Sárdy M, Horváth ON, Goebeler M, Schmidt E, Eming R, Hertl M, Spindler V, Waschke J. Different signaling patterns contribute to loss of keratinocyte cohesion dependent on autoantibody profile in pemphigus. Sci Rep. 2017 Jun 15;7(1):3579. doi: 10.1038/s41598-017-03697-7.
50	111	2013	complications	Italy	20	Clin Dermatol	Journal Article	23806155	Ruocco E, Wolf R, Ruocco V, Brunetti G, Romano F, Lo Schiavo A. Pemphigus: associations and management guidelines: facts and controversies. Clin Dermatol. 2013 Jul-Aug;31(4):382-390. doi: 10.1016/j.clindermatol.2013.01.005.
51	111	2012	administration & dosage	Netherlands	20	Br J Dermatol	Clinical Trial	21967609	Horváth B, Huizinga J, Pas HH, Mulder AB, Jonkman MF. Low-dose rituximab is effective in pemphigus. Br J Dermatol. 2012 Feb;166(2):405-12. doi: 10.1111/j.1365-2133.2011.10663.x. Epub 2012 Jan 9.
52	111	2011	classification	France	20	Clin Dermatol	Journal Article	21679871	Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol. 2011 Jul-Aug;29(4):432-6. doi: 10.1016/j.clindermatol.2011.01.013.
53	179	2011	blood	Germany	20	Am J Pathol	Journal Article	21281804	Rafei D, Müller R, Ishii N, Llamazares M, Hashimoto T, Hertl M, Eming R. IgG autoantibodies against desmocollin 3 in pemphigus sera induce loss of keratinocyte adhesion. Am J Pathol. 2011 Feb;178(2):718-23. doi: 10.1016/j.ajpath.2010.10.016.
54	102	2015	physiology	Germany	19	J Invest Dermatol	Journal Article	26288352	Vielmuth F, Waschke J, Spindler V. Loss of Desmoglein Binding Is Not Sufficient for Keratinocyte Dissociation in Pemphigus. J Invest Dermatol. 2015 Dec;135(12):3068-3077. doi: 10.1038/jid.2015.324. Epub 2015 Aug 19.
55	111	2012	therapeutic use	Germany	19	Clin Dermatol	Journal Article	22137231	Kasperkiewicz M, Schmidt E, Zillikens D. Current therapy of the pemphigus group. Clin Dermatol. 2012 Jan-Feb;30(1):84-94. doi: 10.1016/j.clindermatol.2011.03.014.
56	111	2015	immunology	U.S	18	J Biol Chem	Journal Article	26260795	Chen Y, Chernyavsky A, Webber RJ, Grando SA, Wang PH. Critical Role of the Neonatal Fc Receptor (FcRn) in the Pathogenic Action of Antimitochondrial Autoantibodies Synergizing with Anti-desmoglein Autoantibodies in Pemphigus Vulgaris. J Biol Chem. 2015 Sep 25;290(39):23826-37. doi: 10.1074/jbc.M115.668061. Epub 2015 Aug 10.
57	4	2015	endemic diseases	Brazil	18	J Dermatol	Journal Article	25558948	Aoki V, Rivitti EA, Diaz LA. Update on fogo selvagem, an endemic form of pemphigus foliaceus. J Dermatol. 2015 Jan;42(1):18-26. doi: 10.1111/1346-8138.12675.
58	799	2015	physiology	China	18	Br J Dermatol	Journal Article	24935080	Zhu HQ, Xu RC, Chen YY, Yuan HJ, Cao H, Zhao XQ, Zheng J, Wang Y, Pan M. Impaired function of CD19(+) CD24(hi) CD38(hi) regulatory B cells in patients with pemphigus. Br J Dermatol. 2015 Jan;172(1):101-10. doi: 10.1111/bjd.13192. Epub 2014 Nov 20.
59	166	2014	metabolism	U.S	18	J Invest Dermatol	Journal Article	23657501	Mao X, Li H, Sano Y, Gaestel M, Mo Park J, Payne AS. MAPKAP kinase 2 (MK2)-dependent and -independent models of blister formation in pemphigus vulgaris. J Invest Dermatol. 2014 Jan;134(1):68-76. doi: 10.1038/jid.2013.224. Epub 2013 Jun 27
60	111	2012	blood	Japan	18	J Invest Dermatol	Journal Article	22277941	Ohyama B, Nishifuji K, Chan PT, Kawaguchi A, Yamashita T, Ishii N, Hamada T, Dainichi T, Koga H, Tsuruta D, Amagai M, Hashimoto T. Epitope spreading is rarely found in pemphigus vulgaris by large-scale longitudinal study using desmoglein 2-based swapped molecules. J Invest Dermatol. 2012 Apr;132(4):1158-68. doi: 10.1038/jid.2011.448. Epub 2012 Jan 26
61	156	2012	administration & dosage	Italy	18	J Am Acad Dermatol	Journal Article	22243765	Cianchini G, Lupi F, Masini C, Corona R, Puddu P, De Pit? O. Therapy with rituximab for autoimmune pemphigus: results from a single-center observational study on 42 cases with long-term follow-up. J Am Acad Dermatol. 2012 Oct;67(4):617-22. doi: 10.1016/j.jaad.2011.11.007. Epub 2012 Jan 13.
62	111	2012	classification	Australia	18	Clin Dermatol	Journal Article	22137234	Daniel BS, Hertl M, Werth VP, Eming R, Murrell DF. Severity score indexes for blistering diseases. Clin Dermatol. 2012 Jan-Feb;30(1):108-13. doi: 10.1016/j.clindermatol.2011.03.017.
63	163	2018	metabolism	U.S	17	Front Immunol	Journal Article	29915578	Amber KT, Valdebran M, Grando SA. Non-Desmoglein Antibodies in Patients With Pemphigus Vulgaris. Front Immunol. 2018 Jun 4;9:1190. doi: 10.3389/fimmu.2018.01190. eCollection 2018.
64	111	2016	standards	U.S	17	Adv Ther	Journal Article	27287854	Cholera M, Chainani-Wu N. Management of Pemphigus Vulgaris. Adv Ther. 2016 Jun;33(6):910-58. doi: 10.1007/s12325-016-0343-4. Epub 2016 Jun 10.
65	329	2015	blood	Japan	17	Br J Dermatol	Journal Article	25640111	Ishii N, Teye K, Fukuda S, Uehara R, Hachiya T, Koga H, Tsuchisaka A, Numata S, Ohyama B, Tateishi C, Tsuruta D, Furumura M, Hattori S, Kawakami T, Ohata C, Hashimoto T. Anti-desmocollin autoantibodies in nonclassical pemphigus. Br J Dermatol. 2015 Jul;173(1):59-68. doi: 10.1111/bjd.13711. Epub 2015 May 28.
66	237	2014	chemistry	U.S	17	J Invest Dermatol	Journal Article	24056861	Tucker DK, Stahley SN, Kowalczyk AP. Plakophilin-1 protects keratinocytes from pemphigus vulgaris IgG by forming calcium-independent desmosomes. J Invest Dermatol. 2014 Apr;134(4):1033-1043. doi: 10.1038/jid.2013.401. Epub 2013 Sep 20.
67	111	2013	oxidative stress	Turkey	17	J Eur Acad Dermatol Venereol	Journal Article	22324759	Yesilova Y, Ucmak D, Selek S, Dertlio?lu SB, Sula B, Bozkus F, Turan E. Oxidative stress index may play a key role in patients with pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2013 Apr;27(4):465-7. doi: 10.1111/j.1468-3083.2012.04463.x. Epub 2012 Feb 10.
68	316	2011	pharmacology	Germany	17	Am J Pathol	Journal Article	21864491	Spindler V, Endlich A, Hartlieb E, Vielmuth F, Schmidt E, Waschke J. The extent of desmoglein 3 depletion in pemphigus vulgaris is dependent on Ca(2+)-induced differentiation: a role in suprabasal epidermal skin splitting? Am J Pathol. 2011 Oct;179(4):1905-16. doi: 10.1016/j.ajpath.2011.06.043. Epub 2011 Aug 22.
69	111	2012	statistics & numerical data	Taiwan	17	J Invest Dermatol	Journal Article	21850023	Huang YH, Kuo CF, Chen YH, Yang YW. Incidence, mortality, and causes of death of patients with pemphigus in Taiwan: a nationwide population-based study. J Invest Dermatol. 2012 Jan;132(1):92-7. doi: 10.1038/jid.2011.249. Epub 2011 Aug 18
70	111	2011	diagnosis	India	17	Indian J Dermatol Venereol Leprol	Journal Article	21727691	Kanwar AJ, De D. Pemphigus in India. Indian J Dermatol Venereol Leprol. 2011 Jul-Aug;77(4):439-49. doi: 10.4103/0378-6323.82396.
71	111	2011	metabolism	Japan	17	J Invest Dermatol	Journal Article	21048786	Wada N, Nishifuji K, Yamada T, Kudoh J, Shimizu N, Matsumoto M, Peltonen L, Nagafuchi S, Amagai M. Aire-dependent thymic expression of desmoglein 3, the autoantigen in pemphigus vulgaris, and its role in T-cell tolerance. J Invest Dermatol. 2011 Feb;131(2):410-7. doi: 10.1038/jid.2010.330. Epub 2010 Nov 4
72	389	2017	physiology	Germany	16	Br J Dermatol	Journal Article	28600798	Egu DT, Walter E, Spindler V, Waschke J. Inhibition of p38MAPK signalling prevents epidermal blistering and alterations of desmosome structure induced by pemphigus autoantibodies in human epidermis. Br J Dermatol. 2017 Dec;177(6):1612-1618. doi: 10.1111/bjd.15721. Epub 2017 Nov 14.
73	343	2017	therapy	U.K	16	Br J Dermatol	Journal Article	29192996	Harman KE, Brown D, Exton LS, Groves RW, Hampton PJ, Mohd Mustapa MF, Setterfield JF, Yesudian PD. British Association of Dermatologists' guidelines for the management of pemphigus vulgaris 2017. Br J Dermatol. 2017 Nov;177(5):1170-1201. doi: 10.1111/bjd.15930.
74	265	2017	immunology	China	16	J Invest Dermatol	Journal Article	28647348	Yuan H, Zhou S, Liu Z, Cong W, Fei X, Zeng W, Zhu H, Xu R, Wang Y, Zheng J, Pan M. Pivotal Role of Lesional and Perilesional T/B Lymphocytes in Pemphigus Pathogenesis. J Invest Dermatol. 2017 Nov;137(11):2362-2370. doi: 10.1016/j.jid.2017.05.032. Epub 2017 Jun 22.
75	68	2017	immunology	U.S	16	Cell Rep	Journal Article	28052253	Chen J, Zheng Q, Hammers CM, Ellebrecht CT, Mukherjee EM, Tang HY, Lin C, Yuan H, Pan M, Langenhan J, Komorowski L, Siegel DL, Payne AS, Stanley JR. Proteomic Analysis of Pemphigus Autoantibodies Indicates a Larger, More Diverse, and More Dynamic Repertoire than Determined by B Cell Genetics. Cell Rep. 2017 Jan 3;18(1):237-247. doi: 10.1016/j.celrep.2016.12.013.
76	323	2016	etiology	U.S	16	Exp Dermatol	Comparative Study	27305362	Ahmed AR, Carrozzo M, Caux F, Cirillo N, Dmochowski M, Alonso AE, Gniadecki R, Hertl M, López-Zabalza MJ, Lotti R, Pincelli C, Pittelkow M, Schmidt E, Sinha AA, Sprecher E, Grando SA. Monopathogenic vs multipathogenic explanations of pemphigus pathophysiology. Exp Dermatol. 2016 Nov;25(11):839-846. doi: 10.1111/exd.13106.
77	148	2016	diagnosis	Italy	16	Semin Immunopathol	Journal Article	26597100	Di Zenzo G, Amber KT, Sayar BS, Müller EJ, Borradori L. Immune response in pemphigus and beyond: progresses and emerging concepts. Semin Immunopathol. 2016 Jan;38(1):57-74. doi: 10.1007/s00281-015-0541-1. Epub 2015 Nov 23.
78	149	2015	genetics	U.S	16	Eur J Immunol	Journal Article	25867094	Augusto DG, O'Connor GM, Lobo-Alves SC, Bass S, Martin MP, Carrington M, McVicar DW, Petzl-Erler ML. Pemphigus is associated with KIR3DL2 expression levels and provides evidence that KIR3DL2 may bind HLA-A3 and A11 in vivo. Eur J Immunol. 2015 Jul;45(7):2052-60. doi: 10.1002/eji.201445324. Epub 2015 May 6.
79	111	2014	metabolism	Japan	16	Cell Commun Adhes	Journal Article	25078507	Kitajima Y. 150(th) anniversary series: Desmosomes and autoimmune disease, perspective of dynamic desmosome remodeling and its impairments in pemphigus. Cell Commun Adhes. 2014 Dec;21(6):269-80. doi: 10.3109/15419061.2014.943397. Epub 2014 Jul 31.
80	111	2014	administration & dosage		16	JAMA Dermatol	Journal Article	24500264	Heelan K, Al-Mohammedi F, Smith MJ, Knowles S, Lansang P, Walsh S, Shear NH. Durable remission of pemphigus with a fixed-dose rituximab protocol. JAMA Dermatol. 2014 Jul;150(7):703-8. doi: 10.1001/jamadermatol.2013.6739.
81	111	2014	analysis	Netherlands	16	J Invest Dermatol	Letter	24621791	van der Wier G, Pas HH, Kramer D, Diercks GFH, Jonkman MF. Smaller desmosomes are seen in the skin of pemphigus patients with anti-desmoglein 1 antibodies but not in patients with anti-desmoglein 3 antibodies. J Invest Dermatol. 2014 Aug;134(8):2287-2290. doi: 10.1038/jid.2014.140. Epub 2014 Mar 12.
82	198	2013	therapeutic use	France	16	J Am Acad Dermatol	Comparative Study	23850258	Almugairen N, Hospital V, Bedane C, Duvert-Lehembre S, Picard D, Tronquoy AF, Houivet E, D'incan M, Joly P. Assessment of the rate of long-term complete remission off therapy in patients with pemphigus treated with different regimens including medium- and high-dose corticosteroids. J Am Acad Dermatol. 2013 Oct;69(4):583-8. doi: 10.1016/j.jaad.2013.05.016. Epub 2013 Jul 12.
83	61	2013	metabolism	Japan	16	Kaohsiung J Med Sci	Journal Article	23257250	Kitajima Y. New insights into desmosome regulation and pemphigus blistering as a desmosome-remodeling disease. Kaohsiung J Med Sci. 2013 Jan;29(1):1-13. doi: 10.1016/j.kjms.2012.08.001. Epub 2012 Oct 12.
84	111	2012	therapeutic use	Germany	16	Br J Dermatol	Journal Article	22092243	Behzad M, Möbs C, Kneisel A, Möller M, Hoyer J, Hertl M, Eming R. Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult-to-treat pemphigus vulgaris. Br J Dermatol. 2012 Apr;166(4):844-52. doi: 10.1111/j.1365-2133.2011.10732.x.
85	111	2012	administration & dosage	Germany	16	Clin Dermatol	Journal Article	22137230	Meurer M. Immunosuppressive therapy for autoimmune bullous diseases. Clin Dermatol. 2012 Jan-Feb;30(1):78-83. doi: 10.1016/j.clindermatol.2011.03.013.
86	111	2012	administration & dosage	Germany	16	Br J Dermatol	Journal Article	21910700	Kasperkiewicz M, Shimanovich I, Meier M, Schumacher N, Westermann L, Kramer J, Zillikens D, Schmidt E. Treatment of severe pemphigus with a combination of immunoadsorption, rituximab, pulsed dexamethasone and azathioprine/mycophenolate mofetil: a pilot study of 23 patients. Br J Dermatol. 2012 Jan;166(1):154-60. doi: 10.1111/j.1365-2133.2011.10585.x. Epub 2011 Oct 17.
87	2	2011	enzymology	Germany	16	Ann Anat	Journal Article	21441018	Spindler V, Waschke J. Role of Rho GTPases in desmosomal adhesion and pemphigus pathogenesis. Ann Anat. 2011 May;193(3):177-80. doi: 10.1016/j.aanat.2011.02.003. Epub 2011 Mar 5.
88	60	2018	therapeutic use	France	15	Front Immunol	Journal Article	29706952	Musette P, Bouaziz JD. B Cell Modulation Strategies in Autoimmune Diseases: New Concepts. Front Immunol. 2018 Apr 13;9:622. doi: 10.3389/fimmu.2018.00622. eCollection 2018.
89	303	2015	complications		15	Br J Dermatol	Journal Article	25272088	Parameswaran A, Attwood K, Sato R, Seiffert-Sinha K, Sinha AA. Identification of a new disease cluster of pemphigus vulgaris with autoimmune thyroid disease, rheumatoid arthritis and type I diabetes. Br J Dermatol. 2015 Mar;172(3):729-38. doi: 10.1111/bjd.13433. Epub 2015 Feb 15.
90	167	2014	genetics	Germany	15	Am J Pathol	Journal Article	25010392	Dehner C, Rötzer V, Waschke J, Spindler V. A desmoplakin point mutation with enhanced keratin association ameliorates pemphigus vulgaris autoantibody-mediated loss of cell cohesion. Am J Pathol. 2014 Sep;184(9):2528-36. doi: 10.1016/j.ajpath.2014.05.016. Epub 2014 Jul 8.
91	111	2014	metabolism	Egypt	15	Cell Commun Adhes	Journal Article	24460202	Berika M, Garrod D. Desmosomal adhesion in vivo. Cell Commun Adhes. 2014 Feb;21(1):65-75. doi: 10.3109/15419061.2013.876018.
92	111	2013	physiopathology	Australia	15	JAMA Dermatol	Comparative Study	23925444	Sebaratnam DF, Hanna AM, Chee SN, Frew JW, Venugopal SS, Daniel BS, Martin LK, Rhodes LM, Tan JC, Wang CQ, Welsh B, Nijsten T, Murrell DF. Development of a quality-of-life instrument for autoimmune bullous disease: the Autoimmune Bullous Disease Quality of Life questionnaire. JAMA Dermatol. 2013 Oct;149(10):1186-91. doi: 10.1001/jamadermatol.2013.4972.
93	111	2013	therapeutic use	Iran	15	J Eur Acad Dermatol Venereol	Journal Article	23062214	Chams-Davatchi C, Mortazavizadeh A, Daneshpazhooh M, Davatchi F, Balighi K, Esmaili N, Akhyani M, Hallaji Z, Seirafi H, Mortazavi H. Randomized double blind trial of prednisolone and azathioprine, vs. prednisolone and placebo, in the treatment of pemphigus vulgaris. J Eur Acad Dermatol Venereol. 2013 Oct;27(10):1285-92. doi: 10.1111/j.1468-3083.2012.04717.x. Epub 2012 Oct 15.
94	111	2012	blood	U.S	15	Br J Dermatol	Journal Article	22803659	Funakoshi T, Lunardon L, Ellebrecht CT, Nagler AR, O'Leary CE, Payne AS. Enrichment of total serum IgG4 in patients with pemphigus. Br J Dermatol. 2012 Dec;167(6):1245-53. doi: 10.1111/j.1365-2133.2012.11144.x. Epub 2012 Sep 27.
95	111	2012	biosynthesis	U.S	15	Autoimmunity	Journal Article	22686612	Giordano CN, Sinha AA. Cytokine networks in Pemphigus vulgaris: An integrated viewpoint. Autoimmunity. 2012 Sep;45(6):427-39. doi: 10.3109/08916934.2012.697593. Epub 2012 Jul 13.
96	140	2012	metabolism	U.K	15	J Pathol	Journal Article	22294297	Tsang SM, Brown L, Lin K, Liu L, Piper K, O'Toole EA, Grose R, Hart IR, Garrod DR, Fortune F, Wan H. Non-junctional human desmoglein 3 acts as an upstream regulator of Src in E-cadherin adhesion, a pathway possibly involved in the pathogenesis of pemphigus vulgaris. J Pathol. 2012 May;227(1):81-93. doi: 10.1002/path.3982. Epub 2012 Feb 17.
97	95	2019	adoptive transfer	Germany	14	Front Immunol	Journal Article	31293582	Didona D, Maglie R, Eming R, Hertl M. Pemphigus: Current and Future Therapeutic Strategies. Front Immunol. 2019 Jun 25;10:1418. doi: 10.3389/fimmu.2019.01418. eCollection 2019.
98	157	2016	genetics	Israel	14	PLoS Genet	Journal Article	27148741	Vodo D, Sarig O, Geller S, Ben-Asher E, Olender T, Bochner R, Goldberg I, Nosgorodsky J, Alkelai A, Tatarskyy P, Peled A, Baum S, Barzilai A, Ibrahim SM, Zillikens D, Lancet D, Sprecher E. Identification of a Functional Risk Variant for Pemphigus Vulgaris in the ST18 Gene. PLoS Genet. 2016 May 5;12(5):e1006008. doi: 10.1371/journal.pgen.1006008. eCollection 2016 May.
99	137	2016	metabolism	U.S	14	J Invest Dermatol	Journal Article	26763424	Stahley SN, Warren MF, Feldman RJ, Swerlick RA, Mattheyses AL, Kowalczyk AP. Super-Resolution Microscopy Reveals Altered Desmosomal Protein Organization in Tissue from Patients with Pemphigus Vulgaris. J Invest Dermatol. 2016 Jan;136(1):59-66. doi: 10.1038/JID.2015.353.
100	506	2013	therapeutic use	U.S	14	Exp Dermatol	Journal Article	24433179	Amber KT, Staropoli P, Shiman MI, Elgart GW, Hertl M. Autoreactive T cells in the immune pathogenesis of pemphigus vulgaris. Exp Dermatol. 2013 Nov;22(11):699-704. doi: 10.1111/exd.12229.