Treatment strategies for pemphigus vulgaris in Japan

Expert Opinion on Pharmacotherapy

Volumn 9, Issue 9, 2008, Pages 1519-1530

  Hashimoto, Takashi ^a  

^a KURUME UNIVERSITY SCHOOL OF MEDICINE   (Japan)

Author keywords

Ciclosporin; IVIG; Pemphigus vulgaris; Plasmapheresis; Steroid

Indexed keywords

ANTIINFLAMMATORY AGENT; AZATHIOPRINE; BETAMETHASONE; BULLOUS PEMPHIGOID ANTIGEN 1; CAPTOPRIL; CORTICOSTEROID; CYCLOPHOSPHAMIDE; CYCLOSPORIN; DAPSONE; DESMOCOLLIN; DESMOGLEIN; DESMOPLAKIN; DEXAMETHASONE; ENVOPLAKIN; GOLD DERIVATIVE; IMMUNOGLOBULIN A; IMMUNOGLOBULIN G; IMMUNOSUPPRESSIVE AGENT; METHOTREXATE; METHYLPREDNISOLONE; MIZORIBINE; MYCOPHENOLIC ACID 2 MORPHOLINOETHYL ESTER; PENICILLAMINE; PENICILLIN G; PERIPLAKIN; PLECTIN; PREDNISOLONE; RITUXIMAB; TETRACYCLINE; UNINDEXED DRUG; CD20 ANTIGEN; IMMUNOGLOBULIN; MONOCLONAL ANTIBODY;

AGRANULOCYTOSIS; BLISTER; CHILL; CLINICAL FEATURE; COUGHING; DIABETES MELLITUS; DIARRHEA; DISEASE CLASSIFICATION; DIZZINESS; DRUG DOSE REDUCTION; DRUG ERUPTION; DRUG MEGADOSE; DRUG PULSE THERAPY; DYSPNEA; EOSINOPHILIA; FACE EDEMA; GASTROINTESTINAL SYMPTOM; HEMOLYTIC ANEMIA; HEPATITIS; HUMAN; HYPERLIPIDEMIA; HYPERTENSION; IGA PEMPHIGUS; INFECTION; INTERSTITIAL PNEUMONIA; JAPAN; KIDNEY DYSFUNCTION; LEUKOPENIA; LIVER DISEASE; LOW DRUG DOSE; LYMPHOCYTOPENIA; METHEMOGLOBINEMIA; MOON FACE; MORNING DOSAGE; MOUTH LESION; MYALGIA; NAUSEA; NEPHRITIS; NEUTROPENIA; NONHUMAN; OSTEOPOROSIS; PEMPHIGUS; PEMPHIGUS FOLIACEUS; PEMPHIGUS VULGARIS; PLASMAPHERESIS; PROTEINURIA; REVIEW; SINGLE DRUG DOSE; SKIN DEFECT; SKIN MANIFESTATION; STOMACH ULCER; TREATMENT PLANNING; VOMITING; IMMUNOLOGY; RANDOMIZED CONTROLLED TRIAL; TREATMENT OUTCOME;

ANTI-INFLAMMATORY AGENTS; ANTIBODIES, MONOCLONAL; ANTIGENS, CD20; HUMANS; IMMUNOGLOBULINS, INTRAVENOUS; IMMUNOSUPPRESSIVE AGENTS; JAPAN; PEMPHIGUS; PLASMAPHERESIS; PREDNISOLONE; RANDOMIZED CONTROLLED TRIALS AS TOPIC; TREATMENT OUTCOME;

EID: 46749139507     PISSN: 14656566     EISSN: None     Source Type: Journal    
DOI: 10.1517/14656566.9.9.1519     Document Type: Review

References (102)

1. Koch PJ, Franke WW. Desmosomal cadherins: another growing multigene family of adhesion molecules. Curr Opin Cell Biol 1994;6(5):682-7
2. Amagai M: Adhesion molecules. I. keratinocyte-keratinocyte interactions: cadherins and pemphigus. J Invest Dermatol 1995;104(1):146-53
3. Zillikens D. Acquired skin diseases of hemidesmosomes. J Dermatol Sci 1999;20(2):122-33
4. Robinson ND, Hashimoto T, Amagai M, Chan LS. Continuing medical education: the new pemphigus variants. J Am Acad Dermatol 1999;40(5 Pt 1):649-71
5. Hashimoto T. Skin diseases related to abnormality in desmosomes and hemidesmosomes. J Dermatol Sci 1999;20(2):81-4
6. Amagai M, Klaus-Kovtun V, Stanley JR. Autoantibodies against a novel epithelial cadherin in PV, a disease of cell adhesion. Cell 1991;67(5):869-77
7. Hashimoto T, Ogawa MM, Konohana A, Nishikawa T. Detection of pemphigus vulgaris and pemphigus foliaceus antigens by immunoblot analysis using different antigen sources. J Invest Dermatol 1990;94(3):327-31
8. Hashimoto T, Konohana A, Nishikawa T. Immunoblot assay as an aid to the diagnoses of unclassified cases of pemphigus. Arch Dermatol 1991;127(6):843-7
9. Hashimoto T, Amagai M, Garrod DR. Immunofluorescence and immunoblot studies on the reactivity of pemphigus vulgaris and pemphigus foliaceus sera with desmoglein 3 and desmoglein 1. Epithelial Cell Biol 1995;4(2):63-9
10. Jablonska S, Chorzelski TP, Beutner EH, Chorzelska J. Herpetiform pemphigus, a variable pattern of pemphigus. Int J Dermatol 1975;14(5):353-9
11. Ishii K, Amagai M, Komai A, et al. Desmoglein 1 and desmoglein 3 are the target antigens in herpetiform pemphigus. Arch Dermatol 1999;135(8):943-7
12. Brenner S, Bialy-Golan A, Anhalt GJ. Recognition of pemphigus antigens in drug-induced pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol 1997;36(6 Pt 1):919-23
13. Ishii K, Amagai M, Ohata Y, et al. Development of pemphigus vulgaris in a patient with pemphigus foliaceus: anti-desmoglein antibody profile shift by enzyme-linked immunosorbent assay. J Am Acad Dermatol 2000;42(5 Pt 2):859-91
14. Komai A, Amagai M, Ishii K, et al. 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 2001;144(6):1177-82
15. Anhalt GJ, Kim S-C, Stanley JR, et al. An autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med 1990;323(25):1729-35
16. Oursler JR, Labib RS, Ariss-Abdo L, et al. Human autoantibodies against desmoplakins in paraneoplastic pemphigus. J Clin Invest 1992;89(6):1775-82
17. Hashimoto T, Amagai M, Watanabe K, et al. Characterization of paraneoplastic pemphigus autoantigens by immunoblot analysis. J Invest Dermatol 1995;104(5):829-34
18. Kiyokawa C, Ruhrberg C, Karashima T, et al. Envoplakin and periplakin are components of the paraneoplastic pemphigus antigen complex. J Invest Dermatol 1998;111:1236-8
19. Amagai M, Nishikawa T, Nousari HC, et al. Antibodies against desmoglein 3 (pemphigus vulgaris antigen) are present in sera from patients with paraneoplastic pemphigus and cause acantholysis in vivo in neonatal mice, J Clin Invest 1998;102(4):775-82
20. Ebihara T, Hashimoto T, Iwatsuki K, et al. Autoantigens for IgA anti-intercellular antibodies of intercellular IgA vesiculopustular dermatosis. J Invest Dermatol 1991;97(4):742-5
21. Hashimoto T. Immunopathology of IgA pemphigus. Clin Dermatol 2001;19(6):683-9
22. Hashimoto T, Kiyokawa C, Mori O, et al. Human desmocollin 1 (Dis1) is an autoantigen for subcorneal pustular dermatosis type of IgA pemphigus. J Invest Dermatol 1997;109(2):127-31
23. Karpati S, Amagai M, Liu LW, et al. Identification of desmoglein 1 as an autoantigen in a patient with intraepidermal neutrophilic dermatosis type of IgA pemphigus. Exp Dermatol 2000;9(3):224-8
24. Hashimoto T, Komai A, Futei Y, et al. Detection of IgA autoantibodies to desmogleins by an enzyme-lined immunosorbent assay. Arch Dermatol 2001;137(6):735-8
25. Cholzelski TP, Hashimoto T, Nishikawa T. Unusual acantholytic bullous dermatosis associated with neoplasia and antibodies of IgG and IgA classes against bovine desmocollins I and II. J Am Acad Dermatol 1994;31(2 Pt 2):351-5
26. Miyagawa S, Hashimoto T, Nishikawa T, et al. Atypical pemphigus associated with monoclonal IgA gammopathy. J Am Acad Dermatol 1995;32(2 Pt 2):352-7
27. Hisamatsu Y, Amagai M, Garrid DR, et al. The detection of IgG and IgA autoantibodies to desmocollins 1 - 3 by enzyme-linked immunosorbent assays using baculovirus-expressed proteins, in atypical pemphigus but not in typical pemphigus. Br J Dermatol 2004;151(1):73-83
28. Ogawa MM, Hashimoto T, Nishikawa T, Castro RM. IgG subclasses of intercellular antibodies in Brazilian pemphigus foliaceus-the relationship to complement fixing capability. Clin Exp Dermatol 1989;14(1):29-31
29. Morini JP, Jomaa B, Gorgi Y, et al. Pemphigus foliaceus in young women. An endemic focus in the Sousse area of Tunisia. Arch Dermatol 1993;129(1):69-73
30. Abeu-Velez AM, Beutner EH, Montoya F, et al. Analyses of autoantigens in a new form of endemic pemphigus foliaceus in Colombia. J Am Acad Dermatol 2003;49(4):609-14
31. Abreu-Velez AM, Hashimoto T, Bollag WB, et al. A unique form of endemic pemphigus in northern Colombia. J Am Acad Dermatol 2003;49(4):599-608
32. Amagai M, Hashimoto T, Komai A, et al. Usefulness of enzyme-linked immunosorbent assay (ELISA) using recombinant desmogleins 1 and 3 for serodiagnosis of pemphigus. Br J Dermatol 1999;140(2):351-7
33. Ishii K, Amagai M, Hall RP, et al. Characterization of autoantibodies in pemphigus using antigen-specific enzyme-linked immunosorbent assays with baculovirus-expressed recombinant desmogleins. J Immunol 1997;159(4):2010-7
34. Anhalt GJ, Labib RS, Voorhees JJ, et al. Induction of pemphigus in neonatal mice by passive transfer of IgG from patients with the disease. N Engl J Med 1982;306(20):1189-96
35. Amagai M, Hashimoto T, Shimizu N, Nishikawa T. Absorption of pathogenic autoantibodies by the extracellular domain of PV antigen (Dsg3) produced by baculovirus. J Clin Invest 1994;94(1):59-67
36. Amagai M, Hashimoto T, Green K, et al. Antigen-specific immunoadsorption of pathogenic autoantibodies in pemphigus foliaceus. J Invest Dermatol 1995;104(6):895-901
37. Bystryn JC. Adjuvant therapy of pemphigus. Arch Dermatol 1984;120(7):941-51
38. Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus. An update. Arch Dermatol 1996;132(2):203-12
39. Ratnam KV, Phay KL, Tan CK. Pemphigus therapy with oral prednisolone regimens. A 5-year study. Int J Dermatol 1990;29(5):363-7
40. Fernandes NC, Perez M. Treatment of pemphigus vulgaris and pemphigus foliaceus. Experimence with 71 patients over a 20 year period. Rev Inst Med Trop Sao Paulo 2001;43(1):33-6
41. Mentink LF, MacKenzie MW, Toth GG, et al. Randomized controlled trial of adjuvant oral dexamethasone pulse therapy in pemphigus vulgaris. PEMPULS trial. Arch Dermatol 2006;142(5):570-6
42. Mignogna MD, Lo Muzio L, Ruoppo E, et al. High-dose intravenous pulse methylprednisone in the treatment of severe oropharyngeal pemphigus. A pilot study. J Oral Pathol Med 2002;31(6):339-44
43. Toth GG, Van De Meer JB, Jonkman MF. Dexamethasone pulse therapy in pemphigus. J Eur Acad Dermatol Venereol 2002;16(6):607-11
44. Werth VP. Treatment of pemphigus vulgaris with brief, high-dose intravenous glucocorticoids. Arch Dermatol 1996;132(12):1435-9
45. Chryssomallis F, Dimitriades A, Chaidemenos GC, et al. Steroid-pulse therapy in pemphigus vulgaris long term follow-up. Int J Dermatol 1995;34(6):438-42
46. Femiano F, Gombos F, Scully C. Pemphigus vulgaris with oral involvement. Evaluation of two different systemic corticosteroid therapeutic protocols. J Eur Acad Dermatol Venereol 2002;16(4):353-6
47. Fivenson DP, Breneman DL, Rosen GB, et al. Nicotinamide and tetracycline therapy of bullous pemphigoid. Arch Dermatol 1994;130(6):753-8
48. Liu Z, Diaz LA, Troy JL, et al. A passive transfer model of the organ-specific autoimmune disease, bullous pemphigoid, using antibodies generated against the hemidesmosomal antigen, BP180. J Clin Invest 1993;92(5):2480-8
49. Heaphy MR, Albrecht J, Werth VP: Dapsone as a glucocorticoid-sparing agent in maintenance-phase pemphigus vulgaris. Arch Dermatol 2005;141(6):699-702
50. Pandya AG, Dyke C. Treatment of pemphigus with gold. Arch Dermatol 1998;134(9):1104-7
51. Poulin Y, Perry HO, Muller SA. Pemphigus vulgaris. Results of treatment with gold as a steroid-sparing agent in a series of thirteen patients. J Am Acad Dermatol 1984;11(5 Pt 1):851-7
52. Penneys NS, Eaglstein WH, Frost P. Management of pemphigus with gold compounds. A long-term follow-up report. Arch Dermatol 1976;112(2):185-7
53. Alebotta A, Saenz AM, Gonzalez F, et al. Pemphigus vulgaris. Benefits tetracycline as adjuvant therapy in a series of thirteen patients. Int J Dermatol 1999;38(3):217-21
54. Gaspar ZS, Walkden V, Wojnarowska F. Minocycline is a useful adjuvant therapy for pemphigus. Australas. J Dermatol 1996;37(2):93-5
55. Alpsoy E, Yilmaz E, Basaran E, et al. Is the combination of tetracycline and nicotinamide therapy alone effective in pemphigus? Arch Dermatol 1995;131(11):1339-40
56. Sugita K, Hirokawa H, Izu K, Tokura Y. D-penicillamine-induced pemphigus successfully treated with combination therapy of mizoribine and prednisolone. J Dermatolog Treat 2004;15(4):214-7
57. Aberer W, Wolff-Schreiner EC, Stingl G, et al. Azathioprine in the treatment of pemphigus vulgaris. A long-term follow-up. J Am Acad Dermatol 1987;16(3 Pt 1):527-33
58. Benoit Corven C, Carvalho P, Prost C, et al. Treatment of pemphigus vulgaris by azathioprine and low doses of prednisone (Lever scheme). Ann Dermatol Venereol 2003;130(1 Pt 1):13-5
59. Mourellou O, Chaidemenos GC, Koussidou T, Kapetis E. The treatment of pemphigus vulgaris. Experience with 48 patients seen over an 11-year period. Br J Dermatol 1995;133(1):83-7
60. Anstey ACV, Wakelin S, Reynolds NJ. Guidelines for prescribing azathioprine in dermatology. Br J Dermatol 2004;151(6):1123-32
61. Smith TJ, Bystryn JC. Methotrexate as an adjuvant treatment for pemphigus vulgaris. Arch Dermatol 1999;135(10):5-1276
62. Piamphongsant T. Treatment of pemphigus with corticosteroids and cyclophosphamide. J Dermatol 1979;6(6):369-3
63. Fellner MJ, Katz JM, McCabe JB. Successful use of cyclophosphamide and prednisone for initial treatment of pemphigus vulgaris. Arch Dermatol 1978;114(6):889-94
64. Cummins DL, Mimouni D, Anhalt GJ, et al. Oral cyclophosphamide for treatment of pemphigus vulgaris and foliaceus. J Am Acad Dermatol 2003;49(2):276-80
65. Chrysomallis F, Ioannides D, Teknetzis A, et al. Treatment of oral pemphigus vulgaris. Int J Dermatol 1994;33:803-7
66. Fleischli ME, Valek RH, Pandya AG. Pulse intravenous cyclophosphamide therapy in pemphigus. Arch Dermatol 1999;135(1):57-61
67. Mobini N, Padilla JR, Ahmed AR. Long-term remission in selected patients with pemphigus vulgaris treated with cyclosporine. J Am Acad Dermatol 1997;36(2 Pt 1):264-6
68. Ioannides D, Chrysomallis F, Bystryn JC. Ineffectiveness of cyclosporine as an adjuvant to corticosteroids in the treatment of pemphigus. Arch Dermatol 2000;136(7):868-72
69. Vardy DA, Cohen AD. Cyclosporine therapy should be considered for maintenance of remission in patients with pemphigus. Arch Dermatol 2001;137(4):505-6
70. Mimouni D, Anhalt GJ, Cummins DL, et al. Treatment of pemphigus vulgaris and pemphigus foliaceus with mycophenolate mofetil. Arch Dermatol 2003;139(6):739-42
71. Powell AM, Albert S, Al Fares S, et al. An evaluation of the usefulness of mycophenolate mofetil in pemphigus. Br J Dermatol 2003;149(1):138-45
72. Enk AH, Knop J. Mycophenolate is effective in the treatment of pemphigus vulgaris. Arch Dermatol 1999;135(1):54-6
73. Nousari HC, Anhalt GJ. The role of mycophenolate mofetil in the management of pemphigus. Arch Dermatol 1999;135(7):853-4
74. Enk AH, Knop J. Treatment of pemphigus vulgaris with mycophenolate mofetil. Lancet 1997;350(9076):494
75. Guillaume JC, Roujeau JC, Morel P, et al. Controlled study of plasma exchange in pemphigus. Arch Dermatol 1988;124(11):1659-63
76. Sondergaard K, Carstens J, Zachariae H. The steroid-sparing effect of long-term plasmapheresis in pemphigus. An update Ther Apher 1997;1(2):155-8
77. Roujeau JC, Andre C, Joneau Fabre M, et al. Plasma exchange in pemphigus. Uncontrolled study often patients. Arch Dermatol 1983;119(3):215-21
78. Ruocco V, Astarita C, Pisani M. Plasmapheresis as an alternative or adjunctive therapy in problem cases of pemphigus. Dermatologica 1984;168(5):219-23
79. Blaszczyk M, Chorzelski TP, Jablonska S, et al. Indications for future. Studies on the treatment of pemphigus with plasmapheresis. Arch Dermatol 1989;125(6):843-4
80. Tan-Lim R, Bystryn JC. Effect of plasmapheresis therapy on circulating levels of pemphigus antibodies. J Am Acad Dermatol 1990;22(1):35-40
81. Turner MS, Sutton D, Sauder DN. The use of plasmapheresis and immunosuppression in the treatment of pemphigus vulgaris. J Am Acad Dermatol 2000;43(6):1058-64
82. Li N, Zhao M, Hilario-Vargas J, et al. Complete FcRn dependence for intravenous Ig therapy in autoimmune skin blistering diseases. J Clin Invest 2005;115(12):3440-50
83. Herzog S, Schmidt E, Goebeler M, et al. Serum levels of autoantibodies to desmoglein 3 in patients with therapy-resistant pemphigus vulgaris successfully treated with adjuvant intravenous immunoglobulins. Acta Derm Venereol 2004;84(1):48-52
84. Baum S, Scope A, Barzilai A, et al. The role of IVIg treatment in severe pemphigus vulgaris. J Eur Acad Dermatol Venereol 2006;20(5):548-52
85. Shimanovich I, Nitschke M, Rose C, et al. Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins. Br J Dermatol 2008;158(2):382-8
86. Segura S, Iranzo P, Martínez DE, et al. High-dose intravenous immunoglobulins for the treatment of autoimmune mucocutaneous blistering diseases: evaluation of its use in 19 cases. J Am Acad Dermatol 2007;56(6):960-7
87. Bystryn JC, Jiao D. IVIg selectively and rapidly decreases circulating pathogenic autoantibodies in pemphigus vulgaris. Autoimmunity 2006;39(7):601-7
88. Ahmed AR, Spigelman Z, Cavacini LA, Posner MR. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med 2006;355(17):1772-9
89. Arredondo J, Chernyavsky AI, Karaouni A, Grando SA. Novel mechanisms of target cell death and survival and of therapeutic action of IVIg in Pemphigus. Am J Pathol 2005;167(6):1531-44
90. Mimouni D, Blank M, Ashkenazi L, et al. Protective effect of intravenous immunoglobulin (IVIG) in an experimental model of pemphigus vulgaris. Clin Exp Immunol 2005;142(3):426-32
91. Arin MJ, Engert A, Krieg T, et al. Anti-CD20 monoclonal antibody (rituximab) in the treatment of pemphigus. Br J Dermatol 2005;153(3):620-5
92. Schmidt E, Hunzelmann N, Zillikens D, et al. Rituximab in refractory autoimmune bullous diseases. Clin Exp Dermatol 2006;31(4):503-8
93. Morrison LH. Therapy of refractory pemphigus vulgaris with monoclonal anti-CD20 antibody (rituximab). J Am Acad Dermatol 2004;51(5):817-9
94. Dupuy A, Viguier M, Bedane C, et al. Treatment of refractory pemphigus valgaris with rituximab (anti-CD20 monoclonal antibody). Arch Dermatol 2004;140(1):91-6
95. Pfütze M, Niedermeier A, Hertl M, Eming R. Introducing a novel Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) in pemphigus. Eur J Dermatol 2007;17(1):4-11
96. Jacobi A, Shuler G, Hertl M. Rapid control of therapy-refractory pemphigus vulgaris by treatment with the tumour necrosis factor-alpha inhibitor infliximab. Br J Dermatol 2005;153(2):448-9
97. Pardo J, Mercader P, Mahiques L, et al. Infliximab in the management of severe pemphigus vulgaris. Br J Dermatol 2005;153(1):222-3
98. Lin M, Hsu C, Lee J. Successful treatment of the recalcitrant pemphigus vulgaris and pemphigus vegetans with etanercept and carbon dioxide laser. Arch Dermatol 2005;141(6):680-2
99. Berookhim B, Fischer HD, Weinberg JM. Treatment of recalcitrant pemphigus vulgaris with tumor necrosis factor alpha antagonist etanercept. Cutis 2004;74(4):245-7
100. Grando SA. New approaches to the treatment of pemphigus. J Invest Deramtol Symp Proc 2004;9(1):84-91
101. Iraji F, Yoosefi A. Healing effect of pilocarpine gel 4% on skin lesions of pemphigus vulgaris. Int J Dermatol 2006;45(6):743-6
102. Anhalt GJ, Werth VP, Strober B, et al. An open-label phase I clinical study to assess the safety of PI-0824 in patients with pemphigus vulgaris. J Invest Dermatol 2005;125:1088