Abstract
Background: Pemphigus vulgaris is a rare, chronic, autoimmune mucocutaneous blistering disease. The disease can progress to involve the skin and multiple mucosae. Pemphigus vulgaris can be associated with a high morbidity and significant mortality rate. Treatment of the condition can be challenging. Conventional therapy primarily consists of systemic corticosteroids and immunosuppressant agents. In some patients with pemphigus vulgaris, these agents fail to provide an effective clinical response or have significant adverse effects.
Methods: We evaluated data on 792 patients with pemphigus vulgaris retrieved from PubMed, covering the period 1973–2004. Only patients reported in the English literature were included in this review.
Recently, several new therapeutic agents and treatment modalities have been described for the treatment of patients with pemphigus vulgaris. Some therapeutic agents that were used in the past and abandoned have recently regained favor. This review focuses on the therapeutic uses of dapsone, methotrexate, mycophenolate mofetil, chlorambucil, dexamethasone-cyclophosphamide pulse therapy, immunoablative therapy with cyclophosphamide, plasmapheresis, and extracorporeal photochemotherapy. Newer agents, such as intravenous immunoglobulin (IVIg) therapy and rituximab (an anti-CD20 chimeric monoclonal antibody), are also discussed.
Results and conclusions: Among the oral agents, dapsone may be considered a first-line agent. This is primarily because the risk of potentially fatal adverse effects with this drug is lower than that associated with other available chemotherapeutic agents. In patients who are refractory to oral agents, alternative treatments have been used to prevent further disease progression. Recently, the use of IVIg therapy, with a defined protocol, has been reported to be beneficial. This therapy is promising since it may allow for discontinuation of all other therapies and is safe. The adverse effects from IVIg therapy are minimal. Furthermore, compared with other therapies, it provides a better quality of life.
Similar content being viewed by others
References
Ahmed AR. Lymphocyte studies in pemphigus. Arch Dermatol Res. 1981; 271: 111–5
Simon DG, Krutchkoff D, Kaslow RA, et al. Pemphigus in Hartford County, Connecticut, from 1972 to 1977. Arch Dermatol. 1980; 116: 1035–7
Kanwar AJ, Kaur S. Pemphigus in children. Int J Dermatol. 1991; 30: 343–6
Wananukul S, Pongprasit P. Childhood pemphigus. Int J Dermatol. 1999; 38: 29–35
Park MS, Terasaki PI, Ahmed AR, et al. HLA-DRW4 in 91 % of Jewish pemphigus vulgaris patients. Lancet. 1979; II: 441–2
Alsaleh QA, Nanda A, Al-Baghli NM, et al. Pemphigus in Kuwait. Int J Dermatol. 1999; 38: 351–6
Brautbar C, Moscovitz M, Livshits T, et al. HLA-DRw4 in pemphigus vulgaris patients in Israel. Tissue Antigens. 1980; 16: 238–43
Lever WF, Hashimoto K. The etiology and treatment of pemphigus and pemphigoid. J Invest Dermatol. 1969; 53: 373–89
Jablonska S. Immunopathology of buttons diseases. Ann Clin Res. 1970; 2: 7–12
Sami N, Bhol KC, Ahmed AR. Diagnostic features of pemphigus vulgaris in patients with pemphigus foliaceus: detection of both autoantibodies, long-term follow-up and treatment responses. Clin Exp Immunol. 2001; 125: 492–8
Lenz P, Amagai M, Vole-Platzer B, et al. Desmoglein 3-ELISA: a pemphigus vulgaris-specific diagnostic tool. Arch Dermatol. 1999; 135: 143–8
Lever WF, White H. Treatment of pemphigus with corticosteroids: results obtained in 46 patients over a period of 11 years. Arch Dermatol. 1963; 87: 12–26
Bystryn JC. Adjuvant therapy of pemphigus. Arch Dermatol. 1984; 120: 941–51
Truhan AP, Ahmed AR. Corticosteroids: a review with emphasis on complications of prolonged systemic therapy. Ann Allergy. 1989; 62: 375-91
Bystryn JC, Steinman NM. The adjuvant therapy of pemphigus: an update. Arch Dermatol. 1996; 132: 203–12
Bystryn JC. Therapy of pemphigus. Semin Dermatol. 1988; 7: 186–94
McDonald CJ. Cytotoxic agents for use in dermatology. I. J Am Acad Dermatol. 1985; 12: 753–75
Ahmed AR, Hombal SM. Cyclophosphamide (Cytoxan): a review on relevant pharmacology and clinical uses. J Am Acad Dermatol. 1984; 11: 1115–26
Feltner MJ, Sapadin AN. Current therapy of pemphigus vulgaris. Mt Sinai J Med. 2001; 68: 268–78
Samitz MH, Rosenberg PE. Subcorneal pustular dermatosis [letter]. Arch Dermatol. 1969; 100: 372
Mandel EH, Gonzales V. Subcomeal pustular dermatosis (Sneddon-Wilkinson). Arch Dermatol; 1969; 99: 246–7
Morgan JK, Marsden CW, Coburn JG, et al. Dapsone in dermatitis herpetiformis. Lancet. 1955; 268: 1197–200
Rogers RS, Sheridan PJ, Nightingale SH. Desquamative gingivitis: clinical, histopathologic, immunopathologic, and therapeutic observations. J Am Acad Dermatol; 1982; 7: 729–35
Buhac J, Bhol K, Padilla T, et al. Coexistence of pemphigus vulgaris and ocular cicatricial pemphigoid. J Am Acad Dermatol. 1996; 34: 884–6
Jablonska S, Chorzelski T. When and how to use sulfones in buttons diseases. Int J Dermatol. 1981; 20: 103–5
Barranco VP. Inhibition of lysosomal enzymes by dapsone. Arch Dermatol. 1974; 110: 563–6
Mier PD, van den Hurk JJ. Inhibition of lysosomal enzymes by dapsone. Br J Dermatol. 1975; 93: 471–2
Haim S, Friedman-Birnbaum R. Dapsone in the treatment of pemphigus vulgaris. Dermatologica 1978; 156: 120–3
van Zyl JM, Basson K, Kriegler A, et al. Mechanisms by which clofazimine and dapsone inhibit the myeloperoxidase system: a possible correlation with their anti-inflammatory properties. Biochem Pharmacol. 1991; 42: 599–608
Thuong-Nguyen V, Kadunce DP, Hendrix JD, et al. Inhibition of neutrophil adherence to antibody by dapsone: a possible therapeutic mechanism of dapsone in the treatment of IgA dermatoses. J Invest Dermatol. 1993; 100: 349–55
Coleman MD. Dapsone: modes of action, toxicity and possible strategies for increasing patient tolerance. Br J Dermatol. 1993; 129: 507–13
Fraki JE, Hopsu-Havu VK. Inhibition of human skin proteinases by chloroquine, dapsone and sulfapyridine. Arch Dermatol Res. 1977; 259: 113–5
Stendahl O, Molin L, Dahlgren C. The inhibition of polymorphonuclear leukocyte cytotoxicity by dapsone: a possible mechanism in the treatment of dermatitis herpetiformis. J Clin Invest. 1978; 62: 214–20
Jeffes EW, Kaplan RP, Ahmed AR. Acantholysis produced in vitro with pemphigus serum: hydrocortisone inhibits acantholysis, while dapsone and 6-mercaptopurine do not inhibit acantholysis. J Clin Immunol. 1984; 4: 359–63
Chiang H, Sirois DA, Bielory L. Chronic oral mucosal ulceration in a 54-year-old female. Ann Allergy Asthma Immunol. 2000; 84: 391–5
DeMento FJ, Grover RW. Acantholytic herpetiform dermatitis. Arch Dermatol. 1973; 107: 883–7
Piamphongsant T. Pemphigus controlled by dapsone. Br J Dermatol. 1976; 94: 681–6
Pearson RW, O’Donoghue M, Kaplan SJ. Pemphigus vegetans: its relationship to eosinophilic spongiosis and favorable response to dapsone. Arch Dermatol. 1980; 116: 65–8
Ahmed AR, Salm M. Juvenile pemphigus. J Am Acad Dermatol. 1983; 8799–80
Rodan KP, Hu CH, Nickoloff BJ. Malodorous intertriginous pustules and plaques: pppemphigus vegetans, Hallopeau type. Arch Dermatol. 1987; 123 (3): 393, 396–7
Ahmed AR, Hombal S. Use of cyclophosphamide in azathioprine failures in pemphigus. J Am Acad Dermatol. 1987; 17: 437–42
Barnard GF, Scharf MJ, Dagher RK. Sulfone syndrome in a patient receiving steroids for pemphigus. Am J Gastroenterol. 1994; 89: 2057–9
Bjarnason B, Skoglund C, Flosadottir E. Childhood pemphigus vulgaris treated with dapsone: a case report. Pediatr Dermatol. 1998; 15: 381–3
Tan HH, Tay YK. An unusual case of pemphigus vulgaris presenting as bilateral foot ulcers. Clin Exp Dermatol. 2000; 25: 224–6
Jetton RL. Methotrexate and its uses in dermatology. Med Times. 1970; 98: 83–92
Ashton H, Beveridge GW, Stevenson CJ. Therapeutics: XI. Immunosuppressive drugs. Br J Dermatol. 1970; 83: 326–30
Peck SM. How I treat pemphigus vulgaris. Postgrad Med. 1970; 48: 271–3
Tuffanelli DL. Pemphigus. Clin Pharmacol Ther. 1974; 16: 974–9
Rudolph RI, Leyden JJ. Dermatologic therapy with immunosuppressive agents. Postgrad Med. 1975; 58: 103–8
Lynch PJ, Gallego RE, Saied NK. Pemphigus: a review. Ariz Med. 1976; 33: 1030–7
Adam BA. Buttons diseases in Malaysia: epidemiology and natural history. Int J Dermatol. 1992; 31: 42–5
Peck SM, Osserman KE. Studies in buttons diseases: treatment of pemphigus vulgaris with methotrexate, two patients (one with concurrent myasthenia gravis). J Mt Sinai Hosp N Y. 1969; 36: 71–6
Lever WF, Goldberg HS. Treatment of pemphigus vulgaris with methotrexate. Arch Dermatol. 1969; 100: 70–8
Jablonska S, Chorzelski T, Blaszczyk M. Immunosuppressants in the treatment of pemphigus. Br J Dermatol. 1970; 83: 315–23
Haim S, Shafrir A. Remarks on the treatment of pemphigus vulgaris. Dermatologica. 1970; 141: 270–6
Lever WF. Pemphigus vulgaris. Arch Dermatol. 1970; 102: 114–6
Peck SM, Osserman KE, Samuels AJ, et al. Studies in buttons diseases: treatment of pemphigus vulgaris with immunosuppressives (steroids and methotrexate) and leucovorin calcium. Arch Dermatol. 1971; 103: 141–7
Ryan JG. Pemphigus: a 20-year survey of experience with 70 cases. Arch Dermatol. 1971; 104: 14–20
Lever WF. Methotrexate and prednisone in pemphigus vulgaris: therapeutic results obtained in 36 patients between 1961 and 1970. Arch Dermatol. 1972; 106: 491–7
Piamphongsant T, Sivayathorn A. Pemphigus: combined treatment with methotrexate and prednisone. J Med Assoc Thai. 1975; 58: 171–6
Haim S, Friedman-Bimbaum R. Pyoderma gangrenosum in immunosuppressed patients. Dermatologica. 1976; 153: 44–8
Mashkilleyson N, Mashkilleyson AL. Mucous membrane manifestations of pemphigus vulgaris: a 25-year survey of 185 patients treated with corticosteroids or with combination of corticosteroids with methotrexate or heparin. Acta Derm Venereol. 1988; 68: 413–21
Lever WE Schaumburg-Lever G. Immunosuppressants and prednisone in pemphigus vulgaris: therapeutic results obtained in 63 patients between 1961 and 1975. Arch Dermatol. 1977; 113: 1236–41
Smith TJ, Bystryn JC. Methotrexate as an adjuvant treatment for pemphigus vulgaris. Arch Dermatol. 1999; 135: 1275–6
Guzzo C. Folic acid supplementation with methotrexate therapy: what benefit if any? [letter]. J Am Acad Dermatol. 1994; 31: 689
Enk AH, Knop J. Mycophenolate is effective in the treatment of pemphigus vulgaris. Arch Dermatol. 1999; 135: 54–6
Moore R, Griffin P, Darby C, et al. Mycophenolate mofetil for prevention of acute rejection [letter]. Lancet. 1995; 346: 253
Lynch WS, Roenigk HH. Mycophenolic acid for psoriasis. Arch Dermatol. 1977; 113: 1203–8
Morris RE, Wang J, Blum JR, et al. Immunosuppressive effects of the morpholinoethyl ester of mycophenolic acid (RS-61443) in rat and nonhuman primate recipients of heart allografts. Transplant Proc. 1991; 23: 19–25
Bohm M, Beissert S, Schwarz T, et al. Buttons pemphigoid treated with mycophenolate mofetil [letter]. Lancet. 1997; 349: 541
Ratnam KV. Adjuvant therapy of bullous pemphigoid with mycophenolate mofetil: old drug, new use. Singapore Med. 12001; 42: 482–4
Nousari HC, Sragovich A, Kimyai-Asadi A, et al. Mycophenolate mofetil in autoimmune and inflammatory skin disorders. J Am Acad Dermatol. 1999; 40: 265–8
Enk AH, Knop J. Treatment of pemphigus vulgaris with mycophenolate mofetil [letter]. Lancet. 1997; 350: 494
Grundmann-Kollmann M, Korting HC, Behrens S, et al. Mycophenolate mofetil: a new therapeutic option in the treatment of blistering autoimmune diseases. J Am Acad Dermatol. 1999; 40: 957–60
Grundmann-Kollmann M, Kaskel P, Leiter U, et al. Treatment of pemphigus vulgaris and buttons pemphigoid with mycophenolate mofetil monotherapy. Arch Dermatol. 1999; 135: 724–5
Bredlich RO, Grundmann-Kollmann M, Behrens S, et al. Mycophenolate mofetil monotherapy for pemphigus vulgaris [letter]. Br J Dermatol. 1999; 141: 934
Lipsky JJ. Mycophenolate mofetil. Lancet. 1996; 348: 1357–9
Kitchin JE, Pomeranz MK, Pak G, et al. Rediscovering mycophenolic acid: a review of its mechanism, side effects, and potential uses. J Am Acad Dermatol. 1997; 37: 445–9
Epinette WW, Parker CM, Jones EL, et al. Mycophenolic acid for psoriasis: a review of pharmacology, long-term efficacy, and safety. J Am Acad Dermatol. 1987; 17: 962–71
Nousari HC, Anhalt GJ. The role of mycophenolate mofetil in the management of pemphigus. Arch Dermatol. 1999; 135: 853–4
Bystryn JC. Is mycophenolic acid effective for the treatment of pemphigus?. Arch Dermatol. 1999; 135: 854–5
Popovsky JL, Camisa C. New and emerging therapies for diseases of the oral cavity. Dermatol Clin. 2000; 18: 113–25
Shah N, Green AR, Elgart GW, et al. The use of chlorambucil with prednisone in the treatment of pemphigus. J Am Acad Dermatol. 2000; 42: 85–8
Aeschlimann A, Brackertz D, Kaiser H, et al. Chlorambucil pulse therapy in progressive chronic polyarthritis. Initial results of an open multicenter study [in German]. Z Rheumatol. 1987; 46: 241–4
Snaith ML, Holt JM. Chlorambucil in the treatment of systemic lupus erythematosus. Am Heart J. 1974; 87: 533–5
Sinoway PA, Callen JP. Chlorambucil: an effective corticosteroid-sparing agent for patients with recalcitrant dermatomyositis. Arthritis Rheum. 1993; 36: 319–24
Elliott JH, Ballinger WH. Behcet’s syndrome: treatment with chlorambucil. Trans Am Ophthalmol Soc. 1984; 82: 264–81
Edwards JC, Leandro MJ, Cambridge G. B-Lymphocyte depletion therapy in rheumatoid arthritis and other autoimmune disorders. Biochem Soc Trans. 2002; 30: 824–8
Shvidel L, Klepfish A, Berrebi A. Successful treatment with rituximab for relapsing immune thrombocytopenic purpura (ITP) associated with low-grade non-Hodgkin’s lymphoma. Am J Hematol. 2001; 67: 213–4
Layios N, Van Den Neste E, Jost E, et al. Remission of severe cold agglutinin disease after rituximab therapy. Leukemia. 2001; 15: 187–8
Heizmann M, Itin P, Wernli M, et al. Successful treatment of paraneoplastic pemphigus in follicular NHL with rituximab: report of a case and review of treatment for paraneoplastic pemphigus in NHL and CLL. Am J Hematol. 2001; 66: 142–4
Dupuy A, Viguier M, Bedane C, et al. Treatment of refractory pemphigus vulgaris with rituximab (anti-CD20 monoclonal antibody). Arch Dermatol. 2004; 140: 91–6
Virgolini L, Marzocchi V. Anti-CD20 monoclonal antibody (rituximab) in the treatment of autoimmune diseases: successful result in refractory pemphigus vulgaris: report of a case [letter]. Haematologica. 2003; 88: ELT24
Cooper HL, Healy E, Theaker JM, et al. Treatment of resistant pemphigus vulgaris with an anti-CD20 monoclonal antibody (rituximab). Clin Exp Dermatol. 2003; 28: 366–8
Herrmann G, Hunzelmann N, Engert A. Treatment of pemphigus vulgaris with anti-CD20 monoclonal antibody (rituximab). Br J Dermatol. 2003; 148: 602–3
Salopek TG, Logsetty S, Tredget EE. 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. 2002; 47: 785–8
Arm 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
Schmidt E, Herzog S, Brocker EB. Long-standing remission of recalcitrant juvenile pemphigus vulgaris after adjuvant therapy with rituximab. Br J Dermatol. 2005; 153 (2): 449–51
Morrison LH. Therapy of refractory pemphigus vulgaris with monoclonal anti-CD20 antibody (Rituximab). J Am Acad Dermatol. 2004; 51 (5): 817–9
Espana A, Femandez-Galar M, Lloret P, et al. Long-term complete remission of severe pemphigus vulgaris with monoclonal anti-CD20 antibody therapy and immunophenotype correlations. J Am Acad Dermatol. 2004; 50 (6): 974–6
Pasricha JS, Thanzama J, Khan UK. Intermittent high-dose dexamethasone-cyclophosphamide therapy for pemphigus. Br J Dermatol. 1988; 119: 73–7
Kaur S, Kanwar AJ. Dexamethasone-cyclophosphamide pulse therapy in pemphigus. Int J Dermatol. 1990; 29: 371–4
Feltner MJ, Katz JM, McCabe JB. Successful use of cyclophosphamide and prednisone for initial treatment of pemphigus vulgaris. Arch Dermatol. 1978; 114: 889–94
Cummins DL, Mimouni D, Anhalt GJ, et al. Oral cyclophosphamide for treatment of pemphigus vulgaris and foliaceus. J Am Acad Dermatol. 2003; 49: 276–80
Hietanen J, Salo OP. Pemphigus: an epidemiological study of patients treated in Finnish hospitals between 1969 and 1978. Acta Derm Venereol. 1982; 62: 491–6
Pasricha JS, Das SS. Curative effect of dexamethasone-cyclophosphamide pulse therapy for the treatment of pemphigus vulgaris. Int J Dermatol. 1992; 31: 875–7
Pasricha JS, Khaitan BK, Raman RS, et al. Dexamethasone-cyclophosphamide pulse therapy for pemphigus. Int J Dermatol. 1995; 34: 875–82
Kumrah L, Ramam M, Shah P, et al. Pituitary-adrenal function following dexamethasone-cyclophosphamide pulse therapy for pemphigus. Br J Dermatol. 2001; 145: 944–8
Kanwar AJ, Kaur S, Thami GP. Long-term efficacy of dexamethasone-cyclophosphamide pulse therapy in pemphigus. Dermatology. 2002; 204: 228–31
Pandya AG, Sontheimer RD. Treatment of pemphigus vulgaris with pulse intravenous cyclophosphamide. Arch Dermatol. 1992; 128: 1626–30
Hayag MV, Cohen JA, Kerdel FA. Immunoablative high-dose cyclophosphamide without stem cell rescue in a patient with pemphigus vulgaris. J Am Acad Dermatol. 2000; 43: 1065–9
Ruocco V, Rossi A, Argenziano G, et al. Pathogenicity of the intercellular antibodies of pemphigus and their periodic removal from the circulation by plasmapheresis. Br J Dermatol. 1978; 98: 237–41
Cotterill JA, Barker DJ, Millard LG. Plasma exchange in the treatment of pemphigus vulgaris [letter]. Br J Dermatol. 1978; 98: 243
Meurer M, Braun-Falco O. Plasma exchange in the treatment of pemphigus vulgaris. Br J Dermatol. 1979; 100: 231–2
Auerbach R, Bystryn JC. Plasmapheresis and immunosuppressive therapy: effect on levels of intercellular antibodies in pemphigus vulgaris. Arch Dermatol. 1979; 115: 728–30
Coverton RW, Armstrong RB. Acanthosis nigricans developing in resolving lesions of pemphigus vulgaris. Arch Dermatol. 1982; 118: 115–6
Ruocco V, Astarita C, Pisani M. Plasmapheresis as an alternative or adjunctive therapy in problem cases of pemphigus. Dermatologica. 1984; 168: 219–23
Enter HH, Loffler H, Christophers E. Synchronization of plasmapheresis and pulse cyclophosphamide therapy in pemphigus vulgaris. Arch Dermatol. 1987; 123: 1205–10
Fine JD, Appell ML, Green LK, et al. Pemphigus vulgaris: combined treatment with intravenous corticosteroid pulse therapy, plasmapheresis, and azathioprine. Arch Dermatol. 1988; 124: 236–9
Blaszczyk M, Chorzelski TP, Jablonska S, et al. Indications for future studies on the treatment of pemphigus with plasmapheresis. Arch Dermatol. 1989; 125: 843–4
Tan-Lim R, Bystryn JC. Effect of plasmapheresis therapy on circulating levels of pemphigus antibodies. J Am Acad Dermatol. 1990; 22: 35–40
Sondergaard K, Carstens J, Jorgensen J, et al. The steroid-sparing effect of longterm plasmapheresis in pemphigus. Acta Derm Venereol. 1995; 75: 150–2
Thakur BK, Bernardi DM, Murali MR, et al. Invasive cutaneous aspergillosis complicating immunosuppressive therapy for recalcitrant pemphigus vulgaris. J Am Acad Dermatol. 1998; 38: 488–90
Ogata K, Yasuda K, Matsushita M, et al. Successful treatment of adolescent pemphigus vulgaris by immunoadsorption method. J Dermatol. 1999; 26: 236–9
Yano C, Ishiji T, Kamide R, et al. A case of pemphigus vulgaris successfully treated with single filtration plasmapheresis: a correlation of clinical disease activity with serum antibody levels. J Dermatol. 2000; 27: 380–5
Shapiro M, Jimenez S, Werth VP. Pemphigus vulgaris induced by D-penicillamine therapy in a patient with systemic sclerosis. J Am Acad Dermatol. 2000; 42: 297–9
Turner MS, Sutton D, Sander DN. The use of plasmapheresis and immunosuppression in the treatment of pemphigus vulgaris. J Am Acad Dermatol. 2000; 43: 1058–64
Bystryn JC, Graf MW, Um JW. Regulation of antibody formation by serum antibody: II. Removal of specific antibody by means of exchange transfusion. J Exp Med. 1970; 132: 1279–87
Sams WM, Schur PH. Studies of the antibodies in pemphigoid and pemphigus. J Lab Clin Med. 1973; 82: 249–54
Jones JV. Response to apheresis: problems of assessment in immune disease. Clin Nephrol. 1986; 26 Suppl. 1: S70–5
Hida MTT, Kitatnma M, Iida T, et al. Clinical reports on plasma exchange in the Kidney Center, Tokai University School of Medicine. Tokai J Exp Clin Med. 1987; 12: 61–6
Stanley JR. Pathophysiology and therapy of pemphigus in the 21st century. J Dermatol. 2001; 28: 645–6
Wenz B, Barland P. Therapeutic intensive plasmapheresis. Semin Hematol. 1981; 18: 147–62
Terman DS, Garcia-Rinaldi R, Dannemann B, et al. Specific suppression of antibody rebound after extracorporeal immunoadsorption: I. Comparison of single versus combination chemotherapeutic agents. Clin Exp Immunol. 1978; 34: 32–41
Derksen RH, Schumman HJ, Gmelig Meyling FH, et al. Rebound and overshoot after plasma exchange in humans. J Lab Clin Med. 1984; 104: 35–43
Roujeau JC, Andre C, Joneau Fabre M, et al. Plasma exchange in pemphigus: uncontrolled study of ten patients. Arch Dermatol. 1983; 119: 215–21
Ruocco V. Plasmapheresis and pulse cyclophosphamide therapy in pemphigus vulgaris: a novelty or a reappraisal?. Arch Dermatol. 1988; 124: 1716–8
Bystryn JC. Plasmapheresis therapy of pemphigus. Arch Dermatol. 1988; 124: 1702–4
Guillaume JC, Roujeau JC, Morel P, et al. Controlled study of plasma exchange in pemphigus. Arch Dermatol. 1988; 124: 1659–63
Roujeau JC. Plasmapheresis therapy of pemphigus and buttons pemphigoid. Semin Dermatol. 1988; 7: 195–200
Korman N. Pemphigus. J Am Acad Dermatol. 1988; 18: 1219–38
Owsianowski M, Gollnick H, Siegert W, et al. Successful treatment of chronic graft-versus-host disease with extracorporeal photopheresis. Bone Marrow Transplant. 1994; 14: 845–8
Rook AH, Freundlich B, Jegasothy BV, et al. Treatment of systemic sclerosis with extracorporeal photochemotherapy: results of a multicenter trial. Arch Dermalot. 1992; 128: 337–46
Knobler RM, Graninger W, Lindmaier A, et al. Extracorporeal photochemotherapy for the treatment of systemic lupus erythematosus: a pilot study. Arthritis Rheum. 1992; 35: 319–24
Heald P, Knobler R, LaRoche L. Photoinactivated lymphocyte therapy of cutaneous T-cell lymphoma. Dermatol Clin. 1994; 12: 443–9
Edelson RL. Photopheresis: a new therapeutic concept. Yale J Biol Med. 1989; 62: 565–77
Rook AH, Cohen JH, Lessin SR, et al. Therapeutic applications of photopheresis. Dermatol Clin. 1993; 11: 339–47
Gross M, Bob EE, Millikan LE. Extracorporeal photopheresis. Int J Dermatol. 1994; 33: 407–11
Edelson RL. Photopheresis: a clinically relevant immunobiologic response modifier. Ann N Y Acad Sci. 1991; 636: 154–64
Azana JM, de Misa RF, Harto A, et al. Severe pemphigus foliaceus treated with extracorporeal photochemotherapy. Arch Dermatol. 1997; 133: 287–9
Perotti C, Torretta L, Viarengo G, et al. Feasibility and safety of a new technique of extracorporeal photochemotherapy: experience of 240 procedures. Haematologica. 1999; 84: 237–41
Efferth T, Fabry U, Osieka R. Induction of apoptosis, depletion of glutathione, and DNA damage by extracorporeal photochemotherapy and psoralen with exposure to UV light in vitro. Anticancer Res. 2001; 21: 2777–83
Rook AH, Jegasothy BV, Heald P, et al. Extracorporeal photochemotherapy for drug-resistant pemphigus vulgaris. Ann Intern Med. 1990; 112: 303–5
Rook AH, Heald PW, Nahass GT, et al. Treatment of autoimmune disease with extracorporeal photochemotherapy: pemphigus vulgaris: preliminary report. Yale J Biol Med. 1989; 62: 647–52
Rook AH. Photopheresis in the treatment of autoimmune disease: experience with pemphigus vulgaris and systemic sclerosis. Ann N Y Acad Sci. 1991; 636: 209–16
Liang G, Nahass G, Kerdel FA. Pemphigus vulgaris treated with photopheresis. J Am Acad Dermatol. 1992; 26: 779–80
Gollnick HP, Owsianowski M, Taube KM, et al. Unresponsive severe generalized pemphigus vulgaris successfully controlled by extracorporeal photopheresis. J Am Acad Dermatol. 1993; 28: 122–4
Wollina U, Lange D, Looks A. Short-time extracorporeal photochemotherapy in the treatment of drug-resistant autoimmune buttons diseases. Dermatology. 1999; 198: 140–4
Wolfe JT, Lessin SR, Singh AH, et al. Review of immunomodulation by photopheresis: treatment of cutaneous T-cell lymphoma, autoimmune disease, and allograft rejection. Artif Organs. 1994; 18: 888–97
Imbach P, Barandun S, d’Apuzzo V, et al. High-dose intravenous gammaglobulin for idiopathic thrombocytopenic purpura in childhood. Lancet. 1981; I: 1228–31
Abe T, Kawasugi K. Use of intravenous immunoglobulin in various medical conditions: a Japanese experience. Cancer. 1991; 68: 1454–9
Harman KE, Black MM. High-dose intravenous immune globulin for the treatment of autoimmune blistering diseases: an evaluation of its use in 14 cases. Br J Dermatol. 1999; 140: 865–74
Tappeiner G, Steiner A. High-dosage intravenous gamma globulin: therapeutic failure in pemphigus and pemphigoid. J Am Acad Dermatol. 1989; 20: 684–5
Humbert P, Derancourt C, Aubin F, et al. Effects of intravenous gamma-globulin in pemphigus [letter]. J Am Acad Dermatol. 1990; 22: 326
Messer G, Sizmann N, Feucht H, et al. High-dose intravenous immunoglobulins for immediate control of severe pemphigus vulgaris. Br J Dermatol. 1995; 133: 1014–6
Beckers RC, Brand A, Vermeer BJ, et al. Adjuvant high-dose intravenous gammaglobulin in the treatment of pemphigus and buttons pemphigoid: experience in six patients. Br J Dermatol. 1995; 133: 289–93
Bewley AP, Keefe M. Successful treatment of pemphigus vulgaris by pulsed intravenous immunoglobulin therapy. Br J Dermatol. 1996; 135: 128–9
Wever S, Zillikens D, Brocker EB. Successful treatment of refractory mucosal lesions of pemphigus vulgaris using intravenous gammaglobulin as adjuvant therapy. Br J Dermatol. 1996; 135: 862–3
Colonna L, Cianchini G, Frezzolini A, et al. Intravenous immunoglobulins for pemphigus vulgaris: adjuvant or first choice therapy?. Br J Dermatol. 1998; 138: 1102–3
Jolles S, Hughes J, Rustin M. Therapeutic failure of high-dose intravenous immunoglobulin in pemphigus vulgaris. J Am Acad Dermatol. 1999; 40: 499–500
Engineer L, Norton LA, Ahmed AR. Nail involvement in pemphigus vulgaris. J Am Acad Dermatol. 2000; 43: 529–35
Ahmed AR. Intravenous immunoglobulin therapy in the treatment of patients with pemphigus vulgaris unresponsive to conventional immunosuppressive treatment. J Am Acad Dermatol. 2001; 45: 679–90
Sami N, Qureshi A, Ruocco E, et al. Corticosteroid-sparing effect of intravenous immunoglobulin therapy in patients with pemphigus vulgaris. Arch Dermatol. 2002; 138: 1158–62
Engineer L, Bhol KC, Ahmed AR. Analysis of current data on the use of intravenous immunoglobulins in management of pemphigus vulgaris. J Am Acad Dermatol. 2000; 43: 1049–57
Ahmed AR, Dahl MV. Consensus statement on the use of intravenous immunoglobulin therapy in the treatment of autoimmune mucocutaneous blistering diseases. Arch Dermatol. 2003; 139 (8): 1051–9
Acknowledgments
No sources of funding were used to assist in the preparation of this study. The authors have no conflicts of interest that are directly relevant to the content of this study.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Yeh, S.W., Sami, N. & Ahmed, R.A. Treatment of Pemphigus Vulgaris. Am J Clin Dermatol 6, 327–342 (2005). https://doi.org/10.2165/00128071-200506050-00006
Published:
Issue Date:
DOI: https://doi.org/10.2165/00128071-200506050-00006