Urban legends: Pemphigus vulgaris

Oral Diseases

Volumn 18, Issue 5, 2012, Pages 442-458

  Cirillo, N ^a   Cozzani, E ^b   Carrozzo, M ^c   Grando, S A ^d  

^a UNIVERSITY OF BRISTOL   (United Kingdom)

^b UNIVERSITY OF GENOA   (Italy)

^c NEWCASTLE UNIVERSITY   (United Kingdom)

^d UNIVERSITY OF CALIFORNIA   (United States)

Author keywords

Acantholysis; Apoptosis; Desmogleins; Pemphigus; Rituximab

Indexed keywords

AZATHIOPRINE; CARCINOEMBRYONIC ANTIGEN RELATED CELL ADHESION MOLECULE 6; CASPASE 3; CD2 ANTIGEN; CD36 ANTIGEN; CD37 ANTIGEN; CD40 ANTIGEN; CHOLINERGIC RECEPTOR; COMPLEMENTARY DNA; CYCLOPHOSPHAMIDE; DAPSONE; DESMOCOLLIN; DESMOGLEIN 1; DESMOGLEIN 1 ANTIBODY; DESMOGLEIN 3; DESMOGLEIN 3 ANTIBODY; DEXAMETHASONE; GOLD; IMMUNOGLOBULIN G; INTERCELLULAR ADHESION MOLECULE 1; METHOTREXATE; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; NEOSPORIN; NERVE CELL ADHESION MOLECULE; P CADHERIN; PREDNISONE; RAPAMYCIN; RITUXIMAB; UNINDEXED DRUG; UVOMORULIN;

ACANTHOLYSIS; ADJUVANT THERAPY; ANTIBODY PRODUCTION; ANTIBODY TITER; ANTIGEN BINDING; APOPTOSIS; ARTICLE; BLISTER; CALCIUM CELL LEVEL; CALCIUM METABOLISM; CELL ACTIVATION; CELL ADHESION; CELL DAMAGE; CELL DEATH; CELL GROWTH; CELL MATURATION; CELL MOTILITY; CELL VOLUME; CHILL; COUGHING; DEEP VEIN THROMBOSIS; DISEASE COURSE; DOWN REGULATION; DRUG EFFICACY; DRUG MEGADOSE; ENZYME ACTIVATION; ENZYME PHOSPHORYLATION; ENZYME RELEASE; EPIDERMIS; FACE EDEMA; HUMAN; IMMUNOSUPPRESSIVE TREATMENT; IN VITRO STUDY; IN VIVO STUDY; KERATINOCYTE; KERATODERMA; LONG TERM CARE; LUNG EMBOLISM; MORTALITY; MOUTH MUCOSA; MULTIPLE CYCLE TREATMENT; MUSCLE HYPOTONIA; NAUSEA; NONHUMAN; PATHOPHYSIOLOGY; PEMPHIGUS VULGARIS; PERIPHERAL BLOOD MONONUCLEAR CELL; PHENOTYPE; PNEUMOCYSTOSIS; PREMEDICATION; PRIORITY JOURNAL; PROTEIN DEGRADATION; PROTEIN DOMAIN; PROTEIN EXPRESSION; PROTEIN FUNCTION; PYELONEPHRITIS; RELAPSE; REMISSION; SEPTICEMIA; SIGNAL TRANSDUCTION; STAPHYLOCOCCAL BACTEREMIA; TACHYCARDIA; TREATMENT RESPONSE; UPREGULATION; VOMITING;

ACANTHOLYSIS; ANTIBODIES, MONOCLONAL, MURINE-DERIVED; AUTOANTIGENS; CELL ADHESION MOLECULES; DESMOGLEINS; HUMANS; IMMUNOSUPPRESSIVE AGENTS; PEMPHIGUS; PROTEIN KINASES; UNITED STATES;

EID: 84861996117     PISSN: 1354523X     EISSN: 16010825     Source Type: Journal    
DOI: 10.1111/j.1601-0825.2011.01899.x     Document Type: Article

References (189)

1. Abasq C, Mouquet H, Gilbert D et al (2009). ELISA testing of anti-desmoglein 1 and 3 antibodies in the management of pemphigus. Arch Dermatol 145: 529-535.
2. Aboobaker J, Morar N, Ramdial PK, Hammond MG (2001). Pemphigus in South Africa. Int J Dermatol 40: 115-119.
3. Abreu-Velez AM, Howard MS, Jiao Z et al (2011). Cardiac autoantibodies from patients affected by a new variant of endemic pemphigus foliaceus in Colombia, South America. J Clin Immunol 31: 985-997.
4. Ahmed AR, Spigelman Z, Cavacini LA, Posner MR (2006). Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 355: 1772-1779.
5. Akman A, Uzun S, Alpsoy E (2010). Immunopathologic features of pemphigus in the east Mediterranean region of Turkey: a prospective study. Skinmed 8: 12-16.
6. Allen E, Yu QC, Fuchs E (1996). Mice expressing a mutant desmosomal cadherin exhibit abnormalities in desmosomes, proliferation, and epidermal differentiation. J Cell Biol 133: 1367-1382.
7. Alsaleh QA, Nanda A, Al-Baghli NM, Dvorak R (1999). Pemphigus in Kuwait. Int J Dermatol 38: 351-356.
8. Amagai M, Klaus-Kovtun V, Stanley JR (1991). Autoantibodies against a novel epithelial cadherin in pemphigus vulgaris, a disease of cell adhesion. Cell 67: 869-877.
9. Amagai M, Karpati S, Prussick R, Klaus-Kovtun V, Stanley JR (1992). Autoantibodies against the amino-terminal cadherin-like binding domain of pemphigus vulgaris antigen are pathogenic. J Clin Invest 90: 919-926.
10. Amagai M, Hashimoto T, Shimizu N, Nishikawa T (1994a). Absorption of pathogenic autoantibodies by the extracellular domain of pemphigus vulgaris antigen (Dsg3) produced by baculovirus. J Clin Invest 94: 59-67.
11. Amagai M, Kàrpàti S, Klaus-Kovtun V, Udey MC, Stanley JR (1994b). Extracellular domain of pemphigus vulgaris antigen (desmoglein 3) mediates weak homophilic adhesion. J Invest Dermatol 103: 609-615.
12. Amagai M, Tsunoda K, Zillikens D, Nagai T, Nishikawa T (1999). The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile. J Am Acad Dermatol 40: 167-170.
13. Amagai M, Matsuyoshi N, Wang ZH, Andl C, Stanley JR (2000a). Toxin in bullous impetigo and staphylococcal scalded-skin syndrome targets desmoglein 1. Nat Med 6: 1275-1277.
14. Amagai M, Ahmed AR, Kitajima Y et al (2006). Are desmoglein autoantibodies essential for the immunopathogenesis of pemphigus vulgaris, or just 'witnesses of disease'? Exp Dermatol 15: 815-831.
15. Antonucci A, Negosanti M, Tabanelli M, Varotti C (2007). Treatment of refractory pemphigus vulgaris with anti-CD20 monoclonal antibody (rituximab): five cases. J Dermatolog Treat 18: 178-183.
16. Aoyama Y, Kitajima Y (1999). Pemphigus vulgaris-IgG causes a rapid depletion of desmoglein 3 (Dsg3) from the Triton X-100 soluble pools, leading to the formation of Dsg3-depleted desmosomes in a human squamous carcinoma cell line, DJM-1 cells. J Invest Dermatol 112: 67-71.
17. Aoyama Y, Owada MK, Kitajima Y (1999). A pathogenic autoantibody, pemphigus vulgaris-IgG, induces phosphorylation of desmoglein 3, and its dissociation from plakoglobin in cultured keratinocytes. Eur J Immunol 29: 2233-2240.
18. Arin MJ, Engert A, Krieg T, Hunzelmann N (2005). Anti-CD20 monoclonal antibody (rituximab) in the treatment of pemphigus. Br J Dermatol 153: 620-625.
19. Arkfeld DG (2008). The potential utility of B cell-directed biologic therapy in autoimmune diseases. Rheumatol Int 28: 205-215.
20. Arredondo J, Chernyavsky AI, Karaouni A, Grando SA (2005). Novel mechanisms of target cell death and survival and of therapeutic action of IVIg in pemphigus. Am J Pathol 167: 1531-1544.
21. Arteaga LA, Prisayanh PS, Warren SJ, Liu Z, Diaz LA, Lin MS (2002). A subset of pemphigus foliaceous patients exhibits pathogenic autoantibodies against both desmoglein-1 and desmoglein-3. J Invest Dermatol 118: 806-811.
22. Baroni A, Buommino E, Paoletti I, Orlando M, Ruocco E, Ruocco V (2004). Pemphigus serum and captopril induce heat shock protein 70 and inducible nitric oxide synthase overexpression, triggering apoptosis in human keratinocytes. Br J Dermatol 150: 1070-1080.
23. Bech-Serra JJ, Santiago-Josefat B, Esselens C et al (2006). Proteomic identification of desmoglein 2 and activated leukocyte cell adhesion molecule as substrates of ADAM17 and ADAM10 by difference gel electrophoresis. Mol Cell Biol 26: 5086-5095.
24. Belloni-Fortina A, Faggion D, Pigozzi B et al (2009). Detection of autoantibodies against recombinant desmoglein 1 and 3 molecules in patients with pemphigus vulgaris: correlation with disease extent at the time of diagnosis and during follow-up. Clin Dev Immunol 2009: 187864.
25. Berkowitz P, Hu P, Warren S, Liu Z, Diaz LA, Rubenstein DS (2006). p38MAPK inhibition prevents disease in pemphigus vulgaris mice. Proc Natl Acad Sci U S A 103: 12855-12860.
26. Bermúdez A, Marco F, Conde E, Mazo E, Recio M, Zubizarreta A (2000). Fatal visceral varicella-zoster infection following rituximab and chemotherapy treatment in a patient with follicular lymphoma. Haematologica 85: 894-895.
27. Brandsen R, Frusic-Zlotkin M, Lyubimov H et al (1997). Circulating pemphigus IgG in families of patients with pemphigus: comparison of indirect immunofluorescence, direct immunofluorescence, and immunoblotting. J Am Acad Dermatol 36: 44-52.
28. Browning JL (2006). B cells move to centre stage: novel opportunities for autoimmune disease treatment. Nat Rev Drug Discov 5: 564-576.
29. Bystryn JC, Grando SA (2006). A novel explanation for acantholysis in pemphigus vulgaris: the basal cell shrinkage hypothesis. J Am Acad Dermatol 54: 513-516.
30. Carson PJ, Hameed A, Ahmed AR (1996). Influence of treatment on the clinical course of pemphigus vulgaris. J Am Acad Dermatol 34: 645-652.
31. Chams-Davatchi C, Valikhani M, Daneshpazhooh M et al (2005). Pemphigus: analysis of 1209 cases. Int J Dermatol 44: 470-476.
32. Chams-Davatchi C, Esmaili N, Daneshpazhooh M et al (2007). Randomized controlled open-label trial of four treatment regimens for pemphigus vulgaris. J Am Acad Dermatol 57: 622-628.
33. Chen J, Den Z, Koch PJ (2008). Loss of desmocollin 3 in mice leads to epidermal blistering. J Cell Sci 121: 2844-2849.
34. Chernyavsky AI, Arredondo J, Kitajima Y, Sato-Nagai M, Grando SA (2007). Desmoglein versus non-desmoglein signaling in pemphigus acantholysis: characterization of novel signaling pathways downstream of pemphigus vulgaris antigens. J Biol Chem 282: 13804-13812.
35. Chernyavsky AI, Arredondo J, Piser T, Karlsson E, Grando SA (2008). Differential coupling of M1 muscarinic and alpha7 nicotinic receptors to inhibition of pemphigus acantholysis. J Biol Chem 283: 3401-3408.
36. Chitaev NA, Troyanovsky SM (1997). Direct Ca2+-dependent heterophilic interaction between desmosomal cadherins, desmoglein and desmocollin, contributes to cell-cell adhesion. J Cell Biol 138: 193-201.
37. Cianchini G, Corona R, Frezzolini A, Ruffelli M, Didona B, Puddu P (2007). Treatment of severe pemphigus with rituximab: report of 12 cases and a review of the literature. Arch Dermatol 143: 1033-1038.
38. Cirillo N, Prime SS (2009). Desmosomal interactome in keratinocytes: a systems biology approach leading to an understanding of the pathogenesis of skin disease. Cell Mol Life Sci 66: 3517-3533.
39. Cirillo N, Femiano F, Gombos F, Lanza A (2006). Serum from pemphigus vulgaris reduces desmoglein 3 half-life and perturbs its de novo assembly to desmosomal sites in cultured keratinocytes. FEBS Lett 580: 3276-3281.
40. Cirillo N, Lanza M, Rossiello L, Gombos F, Lanza A (2007a). Defining the involvement of proteinases in pemphigus vulgaris: evidence of matrix metalloproteinase-9 overexpression in experimental models of disease. J Cell Physiol 212: 36-41.
41. Cirillo N, Gombos F, Lanza A (2007b). Pemphigus vulgaris immunoglobulin G can recognize a 130000MW antigen other than desmoglein 3 on peripheral blood mononuclear cell surface. Immunology 121: 377-382.
42. Cirillo N, Femiano F, Gombos F, Lanza A (2007c). Metalloproteinase 9 is the outer executioner of desmoglein 3 in apoptotic keratinocytes. Oral Dis 13: 341-345.
43. Cirillo N, Campisi G, Gombos F, Perillo L, Femiano F, Lanza A (2008a). Cleavage of desmoglein 3 can explain its depletion from keratinocytes in pemphigus vulgaris. Exp Dermatol 17: 858-863.
44. Cirillo N, Lanza M, De Rosa A, Femiano F, Gombos F, Lanza A (2008b). At least three phosphorylation events induced by pemphigus vulgaris sera are pathogenically involved in keratinocyte acantholysis. Int J Immunopathol Pharmacol 21: 189-195.
45. Cirillo N, Lanza A, Gombos F (2009) Pathophysiology of the desmosome. Research Signpost Ed: Kerala, India.
46. Cirillo N, Lanza A, Prime SS (2010). Induction of hyper-adhesion attenuates autoimmune-induced keratinocyte cell-cell detachment and processing of adhesion molecules via mechanisms that involve PKC. Exp Cell Res 316: 580-592.
47. Cirillo N, Prime SS (2011). Keratinocytes synthesize and activate cortisol. J Cell Biochem 11: 1499-1505.
48. Cirillo N, Hassona Y, Pignatelli M, Gasparoto TH, Morgan DJ, Prime SS (2012). Characterization of a novel oral glucocorticoid system and its possible role in disease. J Dent Res 91: 97-103.
49. Cozzani E, Rosa GM, Drosera M, Intra C, Barsotti A, Parodi A (2011). ACE inhibitors can induce circulating antibodies directed to antigens of the superficial epidermal cells. Arch Dermatol Res 303: 327-332.
50. Craythorne E, du Viver A, Mufti GJ, Warnakulasuriya S (2011). Rituximab for the treatment of corticosteroid - refractory pemphigus vulgaris with oral and skin manifestations. J Oral Pathol Med 40: 616-620.
51. Cunha PR, Bystryn JC, Medeiros EP, de Oliveira JR (2006). Sensitivity of indirect immunofluorescence and ELISA in detecting intercellular antibodies in endemic pemphigus foliaceous (Fogo Selvagem). Int J Dermatol 45: 914-918.
52. Davies L, Gray D, Spiller D et al (2009). P53 apoptosis mediator PERP: localization, function and caspase activation in uveal melanoma. J Cell Mol Med 13: 1995-2007.
53. Deb-Basu D, Aleem E, Kaldis P, Felsher DW (2006). CDK2 is required by MYC to induce apoptosis. Cell Cycle 5: 1342-1347.
54. Diaz LA, Arteaga LA, Hilario-Vargas J et al (2004). Anti-desmoglein-1 antibodies in onchocerciasis, leishmaniasis and Chagas disease suggest a possible etiological link to Fogo selvagem. J Invest Dermatol 123: 1045-1051.
55. Ding X, Diaz LA, Fairley JA, Giudice GJ, Liu Z (1999). The anti-desmoglein 1 autoantibodies in pemphigus vulgaris sera are pathogenic. J Invest Dermatol 112: 739-743.
56. Dmochowski M, Hashimoto T, Garrod DR, Nishikawa T (1993). Desmocollins I and II are recognized by certain sera from patients with various types of pemphigus, particularly Brazilian pemphigus foliaceus. J Invest Dermatol 100: 380-384.
57. Dmochowski M, Hashimoto T, Chidgey MA et al (1995). Demonstration of antibodies to bovine desmocollin isoforms in certain pemphigus sera. Br J Dermatol 133: 519-525.
58. Dobrev H, Popova L, Vlashev D (1996). Proteinase inhibitors and pemphigus vulgaris. An in vitro and in vivo study. Arch Dermatol Res 288: 648-655.
59. Emery DJ, Diaz LA, Fairley JA, Lopez A, Taylor AF, Giudice GJ (1995). Pemphigus foliaceus and pemphigus vulgaris autoantibodies react with the extracellular domain of desmoglein-1. J Invest Dermatol 104: 323-328.
60. Eming R, Nagel A, Wolff-Franke S, Podstawa E, Debus D, Hertl M (2008). Rituximab exerts a dual effect in pemphigus vulgaris. J Invest Dermatol 128: 2850-2858.
61. Empinotti JC, Aoki V, Filgueira A et al (2006). Clinical and serological follow-up studies of endemic pemphigus foliaceus (fogo selvagem) in Western Parana, Brazil (2001-2002). Br J Dermatol 155: 446-450.
62. Esaki C, Seishima M, Yamada T, Osada K, Kitajima Y (1995). Pharmacologic evidence for involvement of phospholipase C in pemphigus IgG-induced inositol 1, 4, 5-trisphosphate generation, intracellular calcium increase, and plasminogen activator secretion in DJM-1 cells, a squamous cell carcinoma line. J Invest Dermatol 105: 329-333.
63. Evangelista F, Dasher DA, Diaz LA, Prisayanh PS, Li N (2008). E-cadherin is an additional immunological target for pemphigus autoantibodies. J Invest Dermatol 128: 1710-1718.
64. Farb RM, Dykes R, Lazarus GS (1978). Anti-epidermal-cell-surface pemphigus antibody detaches viable epidermal cells from culture plates by activation of proteinase. Proc Natl Acad Sci U S A 75: 459-463.
65. Faurschou A, Gniadecki R (2008). Two courses of rituximab (anti-CD20 monoclonal antibody) for recalcitrant pemphigus vulgaris. Int J Dermatol 47: 292-294.
66. Feliciani C, Toto P, Wang B, Sauder DN, Amerio P, Tulli A (2003). Urokinase plasminogen activator mRNA is induced by IL-1alpha and TNF-alpha in in vitro acantholysis. Exp Dermatol 12: 466-471.
67. Frusic-Zlotkin M, Pergamentz R, Michel B et al (2005). The interaction of pemphigus autoimmunoglobulins with epidermal cells: activation of the fas apoptotic pathway and the use of caspase activity for pathogenicity tests of pemphigus patients. Ann N Y Acad Sci 1050: 371-379.
68. Frusic-Zlotkin M, Raichenberg D, Wang X, David M, Michel B, Milner Y (2006). Apoptotic mechanism in pemphigus autoimmunoglobulins-induced acantholysis: possible involvement of the EGF receptor. Autoimmunity 39: 563-575.
69. Futei Y, Amagai M, Sekiguchi M, Nishifuji K, Fujii Y, Nishikawa T (2000). Use of domain-swapped molecules for conformational epitope mapping of desmoglein 3 in pemphigus vulgaris. J Invest Dermatol 115: 829-834.
70. Gallo R, Massone C, Parodi A, Guarrera M (2002). Allergic contact dermatitis from thiurams with pemphigus-like autoantibodies. Contact Dermatitis 46: 364-365.
71. García-Carrasco M, Jiménez-Hernández M, Escárcega RO et al (2009). Use of rituximab in patients with systemic lupus erythematosus: an update. Autoimmun Rev 8: 343-348.
72. Getsios S, Huen AC, Green KJ (2004). Working out the strength and flexibility of desmosomes. Nat Rev Mol Cell Biol 5: 271-281.
73. Glennie MJ, French RR, Cragg MS, Taylor RP (2007). Mechanisms of killing by anti-CD20 monoclonal antibodies. Mol Immunol 44: 3823-3837.
74. Gniadecki R, Jemec GB, Thomsen BM, Hansen M (1998). Relationship between keratinocyte adhesion and death: anoikis in acantholytic diseases. Arch Dermatol Res 290: 528-532.
75. Grando SA (1992). Decompensation in proteinase-inhibitor system and application of proteinase inhibitors in pemphigus and pemphigoid. J Dermatol Sci 4: 95-97.
76. Grando SA (2000). Autoimmunity to keratinocyte acetylcholine receptors in pemphigus. Dermatology 201: 290-295.
77. Grando SA (2012). Pemphigus autoimmunity: Hypotheses and realities. Autoimmunity 45: 7-35.
78. Grando SA, Pittelkow MR, Shultz LD, Dmochowski M, Nguyen VT (2001). Pemphigus: an unfolding story. J Invest Dermatol 117: 990-995.
79. Grando SA, Bystryn JC, Chernyavsky AI et al (2009). Apoptolysis: a novel mechanism of skin blistering in pemphigus vulgaris linking the apoptotic pathways to basal cell shrinkage and suprabasal acantholysis. Exp Dermatol 18: 764-770.
80. Grando SA, Laquer VT, Le HM (2011). Sirolimus for acute pemphigus vulgaris: a case report and discussion of dualistic action providing for both immunosuppression and keratinocyte protection. J Am Acad Dermatol 65: 684-686.
81. Gürcan HM, Keskin DB, Stern JN, Nitzberg MA, Shekhani H, Ahmed AR (2009). A review of the current use of rituximab in autoimmune diseases. Int Immunopharmacol 9: 10-25.
82. Hanakawa Y, Schechter NM, Lin C (2002). Molecular mechanisms of blister formation in bullous impetigo and staphylococcal scalded skin syndrome. J Clin Invest 110: 53-60.
83. Harman KE, Albert S, Black MM (2003). Guidelines for the management of pemphigus vulgaris. Br J Dermatol 149: 926-937.
84. Hashimoto K, Shafran KM, Webber PS, Lazarus GS, Singer KH (1983). Anti-cell surface pemphigus autoantibody stimulates plasminogen activator activity of human epidermal cells. A mechanism for the loss of epidermal cohesion and blister formation. J Exp Med 157: 259-272.
85. Hashimoto K, Wun TC, Baird J, Lazarus GS, Jensen PJ (1989). Characterization of keratinocyte plasminogen activator inhibitors and demonstration of the prevention of pemphigus IgG-induced acantholysis by a purified plasminogen activator inhibitor. J Invest Dermatol 92: 310-314.
86. Herbst A, Bystryn JC (2000). Patterns of remission in pemphigus vulgaris. J Am Acad Dermatol 42: 422-427.
87. Hertl M, Zillikens D, Borradori L et al (2008). Recommendations for the use of rituximab (anti-CD20 antibody) in the treatment of autoimmune bullous skin diseases. J Dtsch Dermatol Ges 6: 366-373.
88. Hilario-Vargas J, Dasher DA, Li N et al (2006). Prevalence of anti-desmoglein-3 antibodies in endemic regions of Fogo Selvagem in Brazil. J Invest Dermatol 126: 2044-2048.
89. Hotti A, Järvinen K, Siivola P, Hölttä E (2000). Caspases and mitochondria in c-Myc-induced apoptosis: identification of ATM as a new target of caspases. Oncogene 19: 2354-2362.
90. Huang YH, Kuo CF, Chen YH, Yang YW (2012). Incidence, Mortality, and Causes of Death of Patients with Pemphigus in Taiwan: A Nationwide Population-Based Study. J Invest Dermatol 132: 92-97. August 18. doi:.
91. Hulpiau P, van Roy F (2009). Molecular evolution of the cadherin superfamily. Int J Biochem Cell Biol 41: 349-369.
92. Ihrie RA, Reczek E, Horner JS et al (2003). Perp is a mediator of p53-dependent apoptosis in diverse cell types. Curr Biol 13: 1985-1990.
93. Ihrie RA, Marques MR, Nguyen BT et al (2005). Perp is a p63-regulated gene essential for epithelial integrity. Cell 120: 843-856.
94. Ishii K, Amagai M, Hall RP et al (1997). Characterization of autoantibodies in pemphigus using antigen-specific enzyme-linked immunosorbent assays with baculovirus-expressed recombinant desmogleins. J Immunol 159: 2010-2017.
95. Joly P, Mouquet H, Roujeau JC et al (2007). A single cycle of rituximab for the treatment of severe pemphigus. N Engl J Med 357: 545-552.
96. Kalantari-Dehaghi M, Anhalt G, Camilleri M et al (2011a). Pemphigus vulgaris antibodies target PERP and several other keratinocyte membrane and mitochondrial proteins. J Invest Dermatol 131(Suppl 1): S11.
97. Kalantari-Dehaghi M, Molina DM, Farhadieh M et al (2011b). New targets of pemphigus vulgaris antibodies identified by protein array technology. Exp Dermatol 20: 154-156.
98. Kalish RS (2000). Pemphigus vulgaris: the other half of the story. J Clin Invest 106: 1433-1435.
99. Kalish RS (2001). Possible role for non-desmoglein antigen in pemphigus. J Invest Dermatol 117: 995.
100. Kanwar AJ, Ajith AC, Narang T (2006). Pemphigus in North India. J Cutan Med Surg 10: 21-25.
101. Kasperkiewicz M, Shimanovich I, Ludwig RJ, Rose C, Zillikens D, Schmidt E (2011). Rituximab for treatment-refractory pemphigus and pemphigoid: a case series of 17 patients. J Am Acad Dermatol 65: 552-558.
102. Kasperkiewicz M, Shimanovich I, Meier M et al (2012). 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 166: 154-160.
103. Khandpur S, Sharma VK, Sharma A, Pathria G, Satyam A (2010). Comparison of enzyme-linked immunosorbent assay test with immunoblot assay in the diagnosis of pemphigus in Indian patients. Indian J Dermatol Venereol Leprol 76: 27-32.
104. Kim JH, Kim YH, Kim MR, Kim SC (2011). Clinical efficacy of different doses of rituximab in the treatment of pemphigus: a retrospective study of 27 patients. Br J Dermatol 165: 646-651.
105. Kitajima Y, Aoyama Y, Seishima M (1999). Transmembrane signaling for adhesive regulation of desmosomes and hemidesmosomes, and for cell-cell datachment induced by pemphigus IgG in cultured keratinocytes: involvement of protein kinase C. J Investig Dermatol Symp Proc 4: 137-144.
106. Koch PJ, Mahoney MG, Ishikawa H et al (1997). Targeted disruption of the pemphigus vulgaris antigen (desmoglein 3) gene in mice causes loss of keratinocyte cell adhesion with a phenotype similar to pemphigus vulgaris. J Cell Biol 137: 1091-1102.
107. Komai A, Amagai M, Ishii K et al (2001). The clinical transition between pemphigus foliaceus and pemphigus vulgaris correlates well with the changes in autoantibody profile assessed by an enzyme-linked immunosorbent assay. Br J Dermatol 144: 1177-1182.
108. Korman NJ, Eyre RW, Klaus-Kovtun V, Stanley JR (1989). Demonstration of an adhering-junction molecule (plakoglobin) in the autoantigens of pemphigus foliaceus and pemphigus vulgaris. N Engl J Med 321: 631-635.
109. Kowalczyk AP, Borgwardt JE, Green KJ (1996). Analysis of desmosomal cadherin-adhesive function and stoichiometry of desmosomal cadherin-plakoglobin complexes. J Invest Dermatol 107: 293-300.
110. Kricheli D, David M, Frusic-Zlotkin M et al (2000). The distribution of pemphigus vulgaris-IgG subclasses and their reactivity with desmoglein 3 and 1 in pemphigus patients and their first-degree relatives. Br J Dermatol 143: 337-342.
111. Kwon EJ, Yamagami J, Nishikawa T, Amagai M (2008). Anti-desmoglein IgG autoantibodies in patients with pemphigus in remission. J Eur Acad Dermatol Venereol 22: 1070-1075.
112. Lambert LL, Spriet E, Vandewiele A, Naeyaert JM (2006). Desmoglein 1 and 3 IgG auto-antibody titers do not correlate with pemphigus disease activity in a prospective study. J Invest Dermatol 126: 11.
113. Langan SM, Smeeth L, Hubbard R, Fleming KM, Smith CJ, West J (2008). Bullous pemphigoid and pemphigus vulgaris--incidence and mortality in the UK: population based cohort study. BMJ 337: a180.
114. Lanza A, De Rosa A, Santoro R, Gombos F, Cirillo N (2007). Inhibition of protein phosphorylation, but not synthesis nor lysosomal degradation, prevents keratinocyte adhesion loss induced by pemphigus vulgaris serum. J Stomatol Invest 1: 25-32.
115. Lanza A, Cirillo N, Rossiello R et al (2008). Evidence of key role of Cdk2 overexpression in pemphigus vulgaris. J Biol Chem 283: 8736-8745.
116. Lanza A, Lanza M, Santoro R, Soro V, Prime SS, Cirillo N (2011). Deregulation of PERK in the autoimmune disease pemphigus vulgaris occurs via IgG-independent mechanisms. Br J Dermatol 164: 336-343.
117. Lee HE, Berkowitz P, Jolly PS, Diaz LA, Chua MP, Rubenstein DS (2009). Biphasic activation of p38MAPK suggests that apoptosis is a downstream event in pemphigus acantholysis. J Biol Chem 284: 12524-12532.
118. Lickert H, Bauer A, Kemler R, Stappert J (2000). Casein kinase II phosphorylation of E-cadherin increases E-cadherin/beta-catenin interaction and strengthens cell-cell adhesion. J Biol Chem 275: 5090-5095.
119. Ljubojević S, Lipozencić J, Brenner S, Budimcić D (2002). Pemphigus vulgaris: a review of treatment over a 19-year period. J Eur Acad Dermatol Venereol 16: 599-603.
120. Mahoney MG, Wang Z, Rothenberger K, Koch PJ, Amagai M, Stanley JR (1999). Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. J Clin Invest 103: 461-468.
121. Mamelak AJ, Eid MP, Cohen BA, Anhalt GJ (2007). Rituximab therapy in severe juvenile pemphigus vulgaris. Cutis 80: 335-340.
122. Mao X, Nagler AR, Farber SA et al (2010). Autoimmunity to desmocollin 3 in pemphigus vulgaris. Am J Pathol 177: 2724-2730.
123. Marchenko S, Chernyavsky AI, Arredondo J, Gindi V, Grando SA (2010). Antimitochondrial autoantibodies in pemphigus vulgaris: a missing link in disease pathophysiology. J Biol Chem 285: 3695-3704.
124. Martin LK, Werth VP, Villaneuva EV, Murrell DF (2011). A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol 64: 903-908.
125. Mignogna MD, Fortuna G, Leuci S, Ruoppo E (2010). Oropharyngeal pemphigus vulgaris and clinical remission: a long-term, longitudinal study. Am J Clin Dermatol 11: 137-145.
126. Mimouni D, Foedinger D, Kouba DJ et al (2004). Mucosal dominant pemphigus vulgaris with anti-desmoplakin autoantibodies. J Am Acad Dermatol 51: 62-67.
127. Mimouni D, Bar H, Gdalevich M, Katzenelson V, David M (2010). Pemphigus, analysis of 155 patients. J Eur Acad Dermatol Venereol 24: 947-952.
128. Montagutelli X, Lalouette A, Boulouis HJ, Guénet JL, Sundberg JP (1997). Vesicle formation and follicular root sheath separation in mice homozygous for deleterious alleles at the balding (bal) locus. J Invest Dermatol 109: 324-328.
129. Morioka S, Lazarus GS, Jensen PJ (1987). Involvement of urokinase-type plasminogen activator in acantholysis induced by pemphigus IgG. J Invest Dermatol 89: 474-477.
130. Mouquet H, Musette P, Gougeon ML et al (2008). B-cell depletion immunotherapy in pemphigus: effects on cellular and humoral immune responses. J Invest Dermatol 128: 2859-2869.
131. Mourellou O, Chaidemenos GC, Koussidou T, Kapetis E (1995). The treatment of pemphigus vulgaris. Experience with 48 patients seen over an 11-year period. Br J Dermatol 133: 83-87.
132. Müller E, Kernland K, Caldelari R, Wyder M, Balmer V, Hunziker T (2002). Unusual pemphigus phenotype in the presence of a Dsg1 and Dsg3 autoantibody profile. J Invest Dermatol 118: 551-555.
133. Müller R, Hunzelmann N, Baur V et al (2010). Targeted immunotherapy with rituximab leads to a transient alteration of the IgG autoantibody profile in pemphigus vulgaris. Dermatol Res Pract 2010: 321950.
134. Murrell DF, Dick S, Ahmed AR et al (2008). Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol 58: 1043-1046.
135. Nguyen VT, Lee TX, Ndoye A et al (1998). The pathophysiological significance of nondesmoglein targets of pemphigus autoimmunity. Development of antibodies against keratinocyte cholinergic receptors in patients with pemphigus vulgaris and pemphigus foliaceus. Arch Dermatol 134: 971-980.
136. Nguyen VT, Ndoye A, Shultz LD, Pittelkow MR, Grando SA (2000a). Antibodies against keratinocyte antigens other than desmogleins 1 and 3 can induce pemphigus vulgaris-like lesions. J Clin Invest 106: 1467-1479.
137. Nguyen VT, Ndoye A, Grando SA (2000b). Pemphigus vulgaris antibody identifies pemphaxin. A novel keratinocyte annexin-like molecule binding acetylcholine. J Biol Chem 275: 29466-29476.
138. Nguyen VT, Ndoye A, Grando SA (2000c). Novel human alpha9 acetylcholine receptor regulating keratinocyte adhesion is targeted by Pemphigus vulgaris autoimmunity. Am J Pathol 157: 1377-1391.
139. Nguyen VT, Arredondo J, Chernyavsky AI, Kitajima Y, Pittelkow M, Grando SA (2004). Pemphigus vulgaris IgG and methylprednisolone exhibit reciprocal effects on keratinocytes. J Biol Chem 279: 2135-2146.
140. Ortega Loayza AG, Ramos W, Elgart G et al (2006). Antibodies against desmoglein 1 in healthy subjects in endemic and nonendemic areas of pemphigus foliaceus (fogo selvagem) in Peru. Int J Dermatol 45: 538-542.
141. Osada K, Seishima M, Kitajima Y (1997). Pemphigus IgG activates and translocates protein kinase C from the cytosol to the particulate/cytoskeleton fractions in human keratinocytes. J Invest Dermatol 108: 482-487.
142. Peterson JD, Chan LS (2009). Effectiveness and side effects of anti-CD20 therapy for autoantibody-mediated blistering skin diseases: A comprehensive survey of 71 consecutive patients from the Initial use to 2007. Ther Clin Risk Manag 5: 1-7.
143. Pfütze M, Eming R, Kneisel A, Kuhlmann U, Hoyer J, Hertl M (2009). Clinical and immunological follow-up of pemphigus patients on adjuvant treatment with immuno adsorption or rituximab. Dermatology 218: 237-245.
144. Pretel M, España A, Marquina M, Pelacho B, López-Picazo JM, López-Zabalza MJ (2009). An imbalance in Akt/mTOR is involved in the apoptotic and acantholytic processes in a mouse model of pemphigus vulgaris. Exp Dermatol 18: 771-780.
145. Puviani M, Marconi A, Cozzani E, Pincelli C (2003). Fas ligand in pemphigus sera induces keratinocyte apoptosis through the activation of caspase-8. J Invest Dermatol 120: 164-167.
146. Quartier P, Tournilhac O, Archimbaud C et al (2003). Enteroviral meningoencephalitis after anti-CD20 (rituximab) treatment. Clin Infect Dis 36: e47-e49. Epub 16 January 2003.
147. Renou JP, Bierie B, Miyoshi K et al (2003). Identification of genes differentially expressed in mouse mammary epithelium transformed by an activated beta-catenin. Oncogene 22: 4594-4610.
148. Rickman L, Simrak D, Stevens HP et al (1999). N-terminal deletion in a desmosomal cadherin causes the autosomal dominant skin disease striate palmoplantar keratoderma. Hum Mol Genet 8: 971-976.
149. Ridanpää M, Fodde R, Kielman M (2001). Dynamic expression and nuclear accumulation of beta-catenin during the development of hair follicle-derived structures. Mech Dev 109: 173-181.
150. Risser J, Lewis K, Weinstock MA (2009). Mortality of bullous skin disorders from 1979 through 2002 in the United States. Arch Dermatol 145: 1005-1008.
151. Rodrigues DB, Pereira SA, dos Reis MA et al (2009). In situ detection of inflammatory cytokines and apoptosis in pemphigus foliaceus patients. Arch Pathol Lab Med 133: 97-100.
152. Salopek TG, Logsetty S, Tredget EE (2002). Anti-CD20 chimeric monoclonal antibody (rituximab) for the treatment of recalcitrant, life-threatening pemphigus vulgaris with implications in the pathogenesis of the disorder. J Am Acad Dermatol 47: 785-788.
153. Santiago-Josefat B, Esselens C, Bech-Serra JJ, Arribas J (2007). Post-transcriptional up-regulation of ADAM17 upon epidermal growth factor receptor activation and in breast tumors. J Biol Chem 282: 8325-8331.
154. Schaefer BM, Jaeger CJ, Kramer MD (1996). Plasminogen activator system in pemphigus vulgaris. Br J Dermatol 135: 726-732.
155. Schiltz JR, Michel B, Papay R (1979). Appearance of "pemphigus acantholysis factor" in human skin cultured with pemphigus antibody. J Invest Dermatol 73: 575-581.
156. Schmidt E, Hunzelmann N, Zillikens D, Bröcker EB, Goebeler M (2006). Rituximab in refractory autoimmune bullous diseases. Clin Exp Dermatol 31: 503-508.
157. Schmidt E, Seitz CS, Benoit S, Bröcker EB, Goebeler M (2007). Rituximab in autoimmune bullous diseases: mixed responses and adverse effects. Br J Dermatol 156: 352-356.
158. Schmidt E, Bröcker EB, Goebeler M (2008). Rituximab in treatment-resistant autoimmune blistering skin disorders. Clin Rev Allergy Immunol 34: 56-64.
159. Schmidt E, Goebeler M (2008). Use of CD20- directed therapy in autoimmune skin diseases. Exp Rev Dermatol 3: 259-278.
160. Schmidt E, Gutberlet J, Siegmund D, Berg D, Wajant H, Waschke J (2009a). Apoptosis is not required for acantholysis in pemphigus vulgaris. Am J Physiol Cell Physiol 296: C162-C172.
161. Schmidt E, Hennig K, Mengede C, Zillikens D, Kromminga A (2009b). Immunogenicity of rituximab in patients with severe pemphigus. Clin Immunol 132: 334-341.
162. Schmidt E, Zillikens D (2011). Diagnosis and treatment of patients with autoimmune bullous disorders in Germany. Dermatol Clin 29: 663-671.
163. Schumann H, Baetge J, Tasanen K et al (2000). The shed ectodomain of collagen XVII/BP180 is targeted by autoantibodies in different blistering skin diseases. Am J Pathol 156: 685-695.
164. Scully C, Paes De Almeida O, Porter SR, Gilkes JJ (1999). Pemphigus vulgaris: the manifestations and long-term management of 55 patients with oral lesions. Br J Dermatol 140: 84-89.
165. Seishima M, Esaki C, Osada K, Mori S, Hashimoto T, Kitajima Y (1995). Pemphigus IgG, but not bullous pemphigoid IgG, causes a transient increase in intracellular calcium and inositol 1, 4, 5-triphosphate in DJM-1 cells, a squamous cell carcinoma line. J Invest Dermatol 104: 33-37.
166. Seishima M, Satoh S, Nojiri M, Osada K, Kitajima Y (1997). Pemphigus IgG induces expression of urokinase plasminogen activator receptor on the cell surface of cultured keratinocytes. J Invest Dermatol 109: 650-655.
167. Seishima M, Iwasaki-Bessho Y, Itoh Y, Nozawa Y, Amagai M, Kitajima Y (1999). Phosphatidylcholine-specific phospholipase C, but not phospholipase D, is involved in pemphigus IgG-induced signal transduction. Arch Dermatol Res 291: 606-613.
168. Sekiguchi M, Futei Y, Fujii Y, Iwasaki T, Nishikawa T, Amagai M (2001). Dominant autoimmune epitopes recognized by pemphigus antibodies map to the N-terminal adhesive region of desmogleins. J Immunol 167: 5439-5448.
169. Shamsadini S, Fekri AR, Esfandiarpoor I et al (2006). Determination of survival and hazard functions for pemphigus patients in Kerman, a southern province of Iran. Int J Dermatol 45: 668-671.
170. Sharma VK, Prasad HR, Khandpur S, Kumar A (2006). Evaluation of desmoglein enzyme-linked immunosorbent assay (ELISA) in Indian patients with pemphigus vulgaris. Int J Dermatol 45: 518-522.
171. Shimanovich I, Nitschke M, Rose C, Grabbe J, Zillikens D (2008a). Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins. Br J Dermatol 158: 382-388.
172. Shimanovich I, Nitschke M, Rose C, Grabbe J, Zillikens D (2008b). Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins. Br J Dermatol 158: 382-388.
173. Stanley JR, Yaar M, Hawley-Nelson P, Katz SI (1982). Pemphigus antibodies identify a cell surface glycoprotein synthesized by human and mouse keratinocytes. J Clin Invest 70: 281-288.
174. Stappert J, Kemler R (1994). A short core region of E-cadherin is essential for catenin binding and is highly phosphorylated. Cell Adhes Commun 2: 319-327.
175. Swanson DL, Dahl MV (1983). Methylprednisolone inhibits pemphigus acantholysis in skin cultures. J Invest Dermatol 81: 258-260.
176. Torzecka JD, Narbutt J, Sysa-Jedrzejowska A, Waszczykowska E, Lukamowicz J, Pas HH (2003). Detection of pemphigus autoantibodies by IIF and ELISA tests in patients with pemphigus vulgaris and foliaceus and in healthy relatives. Med Sci Monit 9: CR528-CR533.
177. Torzecka JD, Woźniak K, Kowalewski C et al (2007). Circulating pemphigus autoantibodies in healthy relatives of pemphigus patients: coincidental phenomenon with a risk of disease development? Arch Dermatol Res 299: 239-243.
178. Tsunoda K, Ota T, Aoki M et al (2003). Induction of pemphigus phenotype by a mouse monoclonal antibody against the amino-terminal adhesive interface of desmoglein 3. J Immunol 170: 2170-2178.
179. Uzun S, Durdu M, Akman A et al (2006). Pemphigus in the Mediterranean region of Turkey: a study of 148 cases. Int J Dermatol 45: 523-528.
180. Wang X, Brégégère F, Frusić-Zlotkin M, Feinmesser M, Michel B, Milner Y (2004). Possible apoptotic mechanism in epidermal cell acantholysis induced by pemphigus vulgaris autoimmunoglobulins. Apoptosis 9: 131-143.
181. Warren SJ, Lin MS, Giudice GJ et al (2000). The prevalence of antibodies against desmoglein 1 in endemic pemphigus foliaceus in Brazil. Cooperative Group on Fogo Selvagem Research. N Engl J Med 343: 23-30.
182. Wei J, Chang CY, Chuang YC et al (2004). Heart transplantation at Cheng Hsin General Hospital in Taiwan: 15-year experience. Transplant Proc 36: 2374-2376.
183. Williamson L, Raess NA, Caldelari R et al (2006). Pemphigus vulgaris identifies plakoglobin as key suppressor of c-Myc in the skin. EMBO J 25: 3298-3309.
184. Xue W, Hashimoto K, Toi Y (1998). Functional involvement of urokinase-type plasminogen activator receptor in pemphigus acantholysis. J Cutan Pathol 25: 469-474.
185. Yamamoto Y, Aoyama Y, Shu E, Tsunoda K, Amagai M, Kitajima Y (2007a). Anti-desmoglein 3 (Dsg3) monoclonal antibodies deplete desmosomes of Dsg3 and differ in their Dsg3-depleting activities related to pathogenicity. J Biol Chem 282: 17866-17876.
186. Yamamoto Y, Aoyama Y, Shu E, Tsunoda K, Amagai M, Kitajima Y (2007b). No activation of urokinase plasminogen activator by anti-desmoglein 3 monoclonal IgG antibodies in cultured human keratinocytes. J Dermatol Sci 47: 119-125.
187. Yoshimura T, Seishima M, Nakashima K et al (2001). Increased antibody levels to desmogleins 1 and 3 after administration of carbamazepine. Clin Exp Dermatol 26: 441-445.
188. Zagorodniuk I, Weltfriend S, Shtruminger L et al (2005). A comparison of anti-desmoglein antibodies and indirect immunofluorescence in the serodiagnosis of pemphigus vulgaris. Int J Dermatol 44: 541-544.
189. Zambruno G, Borradori L (2008). Rituximab immunotherapy in pemphigus: therapeutic effects beyond B-cell depletion. J Invest Dermatol 128: 2745-2747.