nach oben

Die Dermatologie

Erschienen in:

18.03.2019 | Pemphigus | Leitthema

Aktuelle Therapie des Pemphigus

verfasst von: N. van Beek, MD, D. Zillikens, E. Schmidt

Erschienen in: Die Dermatologie | Ausgabe 4/2019

Einloggen, um Zugang zu erhalten

Zusammenfassung

Hintergrund

Unter den bullösen Autoimmundermatosen stellt der Pemphigus eine potenziell lebensbedrohliche Erkrankungsgruppe dar. Eine schnell wirksame und effektive Therapie ist daher entscheidend.

Ziel der Arbeit

In dieser Übersichtsarbeit werden die aktuellen Therapien unter Berücksichtigung der Leitlinien erörtert und neue Therapieoptionen vorgestellt.

Methoden

Es erfolgte eine Literaturrecherche.

Ergebnisse

Die Basistherapie des Pemphigus sind systemische Glukokortikosteroide, zudem steht eine Reihe von adjuvanten Immunsuppressiva wie Azathioprin und Mycophenolat-Mofetil/Mycophenolsäure zur Steroideinsparung zur Verfügung. In einer kürzlichen prospektiven randomisierten Studie wurde die Wirkung des Anti-CD20-Antikörpers Rituximab eindrucksvoll gezeigt. In schweren oder refraktären Fällen werden Immunadsorption oder hoch dosierte intravenöse Immunglobuline (IVIG) empfohlen. Eine adjuvante Immunadsorption erscheint auch bei Patienten mit hohen Autoantikörperspiegeln in den ersten 8 bis 12 Wochen der Therapie sinnvoll. Diverse neue Therapeutika befinden sich derzeit in der klinischen Erprobung.

Diskussion

Die Therapie des Pemphigus hat sich durch den Einsatz von Rituximab deutlich verbessert. Hierdurch kann der Einsatz von Glukokortikosteroiden, der mit einer hohen Rate an unerwünschten Nebenwirkungen und erhöhter Mortalität assoziiert war, reduziert werden. Nach einer Zulassung von Rituximab zur Behandlung des Pemphigus durch die amerikanische FDA (Food and Drug Administration) 2018 wird die europäische Zulassung für 2019 erwartet.

Ahmed AR, Shetty S (2015) A comprehensive analysis of treatment outcomes in patients with pemphigus vulgaris treated with rituximab. Autoimmun Rev 14:323–331PubMed

Amagai M, Ikeda S, Shimizu H et al (2009) A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol 60:595–603PubMed

Amber KT, Lamberts A, Solimani F et al (2017) Determining the incidence of pneumocystis pneumonia in patients with autoimmune blistering diseases not receiving routine prophylaxis. Jama Dermatol 153:1137–1141PubMedPubMedCentral

Anhalt G, Strober B, Connolly M, Korman N, Greenstein D, Fantasia J, Kalish R (2005) An open-label phase I clinical study to assess the safety of PI-0824 in patients with pemphigus vulgaris. J Invest Dermatol 125:Abs23

Anhalt GJ (2004) Paraneoplastic pemphigus. J Investig Dermatol Symp Proc 9:29–33PubMed

Aoki-Ota M, Kinoshita M, Ota T et al (2006) Tolerance induction by the blockade of CD40/CD154 interaction in pemphigus vulgaris mouse model. J Invest Dermatol 126:105–113PubMed

Aoyama Y (2010) What’s new in i.v. immunoglobulin therapy and pemphigus: high-dose i.v. immunoglobulin therapy and its mode of action for treatment of pemphigus. J Dermatol 37:239–245PubMed

Baskan EB, Yilmaz M, Tunali S et al (2009) Efficacy and safety of long-term mycophenolate sodium therapy in pemphigus vulgaris. J Eur Acad Dermatol Venereol 23:1432–1434PubMed

Baum S, Debby A, Gilboa S et al (2016) Efficacy of Dapsone in the treatment of pemphigus vulgaris: a single-center case study. Dermatology 232:578–585PubMed

10.

Baum S, Greenberger S, Samuelov L et al (2012) Methotrexate is an effective and safe adjuvant therapy for pemphigus vulgaris. Eur J Dermatol 22:83–87PubMed

11.

Behzad M, Mobs C, Kneisel A et al (2012) Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult-to-treat pemphigus vulgaris. Br J Dermatol 166:844–852PubMed

12.

Beissert S, Mimouni D, Kanwar AJ et al (2010) Treating pemphigus vulgaris with prednisone and mycophenolate mofetil: a multicenter, randomized, placebo-controlled trial. J Invest Dermatol 130:2041–2048PubMed

13.

Beissert S, Werfel T, Frieling U et al (2006) A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus. Arch Dermatol 142:1447–1454PubMed

14.

Berkowitz P, Hu P, Warren S et al (2006) p38MAPK inhibition prevents disease in pemphigus vulgaris mice. Proc Natl Acad Sci U S A 103:12855–12860PubMedPubMedCentral

15.

Bertram F, Brocker EB, Zillikens D et al (2009) Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges 7:434–440PubMed

16.

Bvba A (2017) An open-label, non-controlled, phase II study to evaluate the safety, pharmacodynamics, pharmacokinetics, efficacy and conditions of use of ARGX-113 in patients with mild to moderate pemphigus (vulgaris and foliaceus). clinicaltrials.gov: NCT03334058

17.

Bystryn JC, Steinman NM (1996) The adjuvant therapy of pemphigus. An update. Arch Dermatol 132:203–212PubMed

18.

Caplan A, Fett N, Rosenbach M et al (2017) Prevention and management of glucocorticoid-induced side effects: a comprehensive review: a review of glucocorticoid pharmacology and bone health. J Am Acad Dermatol 76:1–9PubMed

19.

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 57:622–628PubMed

20.

Chams-Davatchi C, Mortazavizadeh A, Daneshpazhooh M et al (2013) Randomized double blind trial of prednisolone and azathioprine, vs. prednisolone and placebo, in the treatment of pemphigus vulgaris. J Eur Acad Dermatol Venereol 27:1285–1292PubMed

21.

Cianchini G, Lupi F, Masini C et al (2012) 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 67:617–622PubMed

22.

Cohen SN, Lim RP, Paul CJ et al (2006) Equal efficacy of topical tacrolimus and clobetasone butyrate in pemphigus foliaceus. Int J Dermatol 45:1379PubMed

23.

Colliou N, Picard D, Caillot F et al (2013) Long-term remissions of severe pemphigus after rituximab therapy are associated with prolonged failure of desmoglein B cell response. Sci Transl Med 5:175ra130

24.

Craythorne EE, Mufti G, Duvivier AW (2011) Rituximab used as a first-line single agent in the treatment of pemphigus vulgaris. J Am Acad Dermatol 65:1064–1065PubMed

25.

De Simone C, Caldarola G, Perino F et al (2012) Enteric-coated mycophenolate sodium as a steroid-sparing agent in pemphigus treatment: a retrospective study. Dermatol Ther 25:219–222PubMed

26.

Dick SE, Werth VP (2006) Pemphigus: a treatment update. Autoimmunity 39:591–599PubMed

27.

Ellebrecht CT, Bhoj VG, Nace A et al (2016) Reengineering chimeric antigen receptor T cells for targeted therapy of autoimmune disease. Science 353:179–184PubMedPubMedCentral

28.

Ellebrecht CT, Choi EJ, Allman DM et al (2014) Subcutaneous veltuzumab, a humanized anti-CD20 antibody, in the treatment of refractory pemphigus vulgaris. Jama Dermatol 150:1331–1335PubMedPubMedCentral

29.

Eming R, Hennerici T, Backlund J et al (2014) Pathogenic IgG antibodies against desmoglein 3 in pemphigus vulgaris are regulated by HLA-DRB1*04:02-restricted T cells. J Immunol 193:4391–4399PubMed

30.

Eming R, Nagel A, Wolff-Franke S et al (2008) Rituximab exerts a dual effect in pemphigus vulgaris. J Invest Dermatol 128:2850–2858PubMed

31.

Eming R, Rech J, Barth S et al (2006) Prolonged clinical remission of patients with severe pemphigus upon rapid removal of desmoglein-reactive autoantibodies by immunoadsorption. Dermatology 212:177–187PubMed

32.

Eming R, Sticherling M, Hofmann SC et al (2015) S2k guidelines for the treatment of pemphigus vulgaris/foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges 13:833–844PubMed

33.

Fardet L, Flahault A, Kettaneh A et al (2007) Corticosteroid-induced clinical adverse events: frequency, risk factors and patient’s opinion. Br J Dermatol 157:142–148PubMed

34.

Feldman RJ, Ahmed AR (2011) Relevance of rituximab therapy in pemphigus vulgaris: analysis of current data and the immunologic basis for its observed responses. Expert Rev Clin Immunol 7:529–541PubMed

35.

Fitzgerald JC, Weiss SL, Maude SL et al (2017) Cytokine Release Syndrome after chimeric antigen receptor T cell therapy for acute lymphoblastic leukemia. Crit Care Med 45:e124–e131PubMedPubMedCentral

36.

Grossman JM, Gordon R, Ranganath VK et al (2010) American College of Rheumatology 2010 recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis. Arthritis Care Res (hoboken) 62:1515–1526

37.

Gurcan HM, Ahmed AR (2009) Analysis of current data on the use of methotrexate in the treatment of pemphigus and pemphigoid. Br J Dermatol 161:723–731PubMed

38.

Gurcan HM, Ahmed AR (2009) Efficacy of dapsone in the treatment of pemphigus and pemphigoid: analysis of current data. Am J Clin Dermatol 10:383–396PubMed

39.

Hahn-Ristic K, Rzany B, Amagai M et al (2002) Increased incidence of pemphigus vulgaris in southern Europeans living in Germany compared with native Germans. J Eur Acad Dermatol Venereol 16:68–71PubMed

40.

Hammers CM, Stanley JR (2016) Mechanisms of disease: Pemphigus and Bullous Pemphigoid. Annu Rev Pathol 11:175–197PubMedPubMedCentral

41.

Harman KE, Brown D, Exton LS et al (2017) British Association of Dermatologists’ guidelines for the management of pemphigus vulgaris 2017. Br J Dermatol 177:1170–1201PubMed

42.

Herrmann G, Hunzelmann N, Engert A (2003) Treatment of pemphigus vulgaris with anti-CD20 monoclonal antibody (rituximab). Br J Dermatol 148:602–603PubMed

43.

Hertl M, Jedlickova H, Karpati S et al (2015) 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 29:405–414PubMed

44.

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 6:366–373PubMed

45.

Heupel WM, Muller T, Efthymiadis A et al (2009) Peptides targeting the Desmoglein 3 adhesive interface prevent autoantibody-induced acantholysis in pemphigus. J Biol Chem 284:8589–8595PubMedPubMedCentral

46.

Hofrichter M, Dworschak J, Emtenani S et al (2018) Immunoadsorption of Desmoglein-3-specific IgG abolishes the blister-inducing capacity of pemphigus vulgaris IgG in neonatal mice. FrontImmunol 9:1935

47.

Horvath B, Huizinga J, Pas HH et al (2012) Low-dose rituximab is effective in pemphigus. Br J Dermatol 166:405–412PubMed

48.

Huang A, Madan RK, Levitt J (2016) Future therapies for pemphigus vulgaris: Rituximab and beyond. J Am Acad Dermatol 74:746–753PubMed

49.

Hubner F, Kasperkiewicz M, Zillikens D et al (2018) Immunoadsorption in dermatology. Hautarzt 70:51–63

50.

Hubner F, Recke A, Zillikens D et al (2016) Prevalence and age distribution of Pemphigus and Pemphigoid diseases in Germany. J Invest Dermatol 136:2495–2498PubMed

51.

Huscher D, Thiele K, Gromnica-Ihle E et al (2009) Dose-related patterns of glucocorticoid-induced side effects. Ann Rheum Dis 68:1119–1124PubMed

52.

Inc. PB (2016) An open-label, phase 2, pilot study investigating the safety, clinical activity, pharmacokinetics, and pharmacodynamics of oral treatment with the BTK inhibitor PRN1008 in patients with newly diagnosed or relapsing pemphigus vulgaris. clinicaltrials.gov

53.

Ioannides D, Apalla Z, Lazaridou E et al (2012) Evaluation of mycophenolate mofetil as a steroid-sparing agent in pemphigus: a randomized, prospective study. J Eur Acad Dermatol Venereol 26:855–860PubMed

54.

Joly P, Maho-Vaillant M, Prost-Squarcioni C et al (2017) 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 389:2031–2040PubMed

55.

Joly P, Mouquet H, Roujeau JC et al (2007) A single cycle of rituximab for the treatment of severe pemphigus. N Engl J Med 357:545–552PubMed

56.

Kanwar AJ, Vinay K (2012) Rituximab in pemphigus. Indian J Dermatol Venereol Leprol 78:671–676PubMed

57.

Kanwar AJ, Vinay K, Sawatkar GU et al (2014) Clinical and immunological outcomes of high- and low-dose rituximab treatments in patients with pemphigus: a randomized, comparative, observer-blinded study. Br J Dermatol 170:1341–1349PubMed

58.

Kasperkiewicz M, Ellebrecht CT, Takahashi H et al (2017) Pemphigus. Nat Rev Dis Primers 3:17026PubMedPubMedCentral

59.

Kasperkiewicz M, Eming R, Behzad M et al (2011) Efficacy and safety of rituximab in pemphigus: experience of the German Registry of Autoimmune Diseases. J Dtsch Dermatol Ges 10:727–732

60.

Kasperkiewicz M, Schmidt E, Zillikens D (2012) Current therapy of the pemphigus group. Clin Dermatol 30:84–94PubMed

61.

Kasperkiewicz M, Shimanovich I, Ludwig RJ et al (2011) Rituximab for treatment-refractory pemphigus and pemphigoid: a case series of 17 patients. J Am Acad Dermatol 65:552–558PubMed

62.

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 166:154–160PubMed

63.

Kim JDB, Werth VP, Joly P, Murrell DF (2015) The treatment of pemphigus vulgaris and pemphigus foliaceus. In: Murrell D (Hrsg) Blistering diseases: clinical features, pathogenesis, treatment. Springer, Berlin Heidelberg, S 523–531

64.

Kridin K (2018) Emerging treatment options for the management of pemphigus vulgaris. Ther Clin Risk Manag 14:757–778PubMedPubMedCentral

65.

Langenhan J, Dworschak J, Saschenbrecker S et al (2014) Specific immunoadsorption of pathogenic autoantibodies in pemphigus requires the entire ectodomains of desmogleins. Exp Dermatol 23:253–259PubMed

66.

Leger S, Picard D, Ingen-Housz-Oro S et al (2012) Prognostic factors of paraneoplastic pemphigus. Arch Dermatol 148:1165–1172PubMed

67.

Lunardon L, Payne AS (2012) Inhibitory human antichimeric antibodies to rituximab in a patient with pemphigus. J Allergy Clin Immunol 130:800–803PubMedPubMedCentral

68.

Martin LK, Werth VP, Villaneuva EV et al (2011) A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol 64:903–908PubMed

69.

Meggitt SJ, Anstey AV, Mohd Mustapa MF et al (2011) British Association of Dermatologists’ guidelines for the safe and effective prescribing of azathioprine 2011. Br J Dermatol 165:711–734PubMed

70.

Meurer M (2012) Immunosuppressive therapy for autoimmune bullous diseases. Clin Dermatol 30:78–83PubMed

71.

Meyersburg D, Schmidt E, Kasperkiewicz M et al (2012) Immunoadsorption in dermatology. Ther Apher Dial 16:311–320PubMed

72.

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 58:1043–1046PubMedPubMedCentral

73.

Murrell DF, Pena S, Joly P et al (2018) Diagnosis and management of pemphigus: recommendations by an international panel of experts. J Am Acad Dermatol https://doi.org/10.1016/j.jaad.2018.02.021 CrossRefPubMed

74.

Nagel A, Podstawa E, Eickmann M et al (2009) Rituximab mediates a strong elevation of B‑cell-activating factor associated with increased pathogen-specific IgG but not autoantibodies in pemphigus vulgaris. J Invest Dermatol 129:2202–2210PubMed

75.

Nousari HC, Anhalt GJ (1999) The role of mycophenolate mofetil in the management of pemphigus. Arch Dermatol 135:853–854PubMed

76.

Orvis AK, Wesson SK, Breza TS Jr. et al (2009) Mycophenolate mofetil in dermatology. J Am Acad Dermatol 60:183–199 (quiz 200–182)PubMed

77.

Parmar NV, Kanwar AJ, Minz RW et al (2013) Assessment of the therapeutic benefit of dexamethasone cyclophosphamide pulse versus only oral cyclophosphamide in phase II of the dexamethasone cyclophosphamide pulse therapy: a preliminary prospective randomized controlled study. Indian J Dermatol Venereol Leprol 79:70–76PubMed

78.

Pollmann R, Schmidt T, Eming R et al (2018) Pemphigus: a comprehensive review on pathogenesis, clinical presentation and novel therapeutic approaches. Clin Rev Allergy Immunol 54:1–25PubMed

79.

Roche H‑L (2015) A randomized, double-blind, double-dummy, active-Comparator, multicenter study to evaluate the efficacy and safety of Rituximab versus MMF in patients with pemphigus vulgaris. clinicaltrials.gov: NCT02383589

80.

Sandborn WJ (2010) State-of-the-art: immunosuppression and biologic therapy. Dig Dis 28:536–542PubMed

81.

Schaeffeler E, Fischer C, Brockmeier D et al (2004) Comprehensive analysis of thiopurine S‑methyltransferase phenotype-genotype correlation in a large population of German-Caucasians and identification of novel TPMT variants. Pharmacogenetics 14:407–417PubMed

82.

Schmidt E (2017) Rituximab as first-line treatment of pemphigus. Lancet 389:1956–1958PubMed

83.

Schmidt E, Brocker EB, Goebeler M (2008) Rituximab in treatment-resistant autoimmune blistering skin disorders. Clin Rev Allergy Immunol 34:56–64PubMed

84.

Schmidt E, Dahnrich C, Rosemann A et al (2010) Novel ELISA systems for antibodies to desmoglein 1 and 3: correlation of disease activity with serum autoantibody levels in individual pemphigus patients. Exp Dermatol 19:458–463PubMed

85.

Schmidt E, Goebeler M (2008) CD20-directed therapy in autoimmune diseases involving the skin: role of rituximab. Expert Rev Dermatol 3:259–278

86.

Schmidt E, Goebeler M, Hertl M et al (2015) S2k guideline for the diagnosis of pemphigus vulgaris/foliaceus and bullous pemphigoid. J Dtsch Dermatol Ges 13:713–727PubMed

87.

Schmidt E, Goebeler M, Zillikens D (2009) Rituximab in severe pemphigus. Ann N Y Acad Sci 1173:683–691PubMed

88.

Schmidt E, Hennig K, Mengede C et al (2009) Immunogenicity of rituximab in patients with severe pemphigus. Clin Immunol 132:334–341PubMed

89.

Schmidt E, Herzog S, Brocker EB et al (2005) Long-standing remission of recalcitrant juvenile pemphigus vulgaris after adjuvant therapy with rituximab. Br J Dermatol 153:449–451PubMed

90.

Schmidt E, Hunzelmann N, Zillikens D et al (2006) Rituximab in refractory autoimmune bullous diseases. Clin Exp Dermatol 31:503–508PubMed

91.

Schmidt E, Klinker E, Opitz A et al (2003) Protein A immunoadsorption: a novel and effective adjuvant treatment of severe pemphigus. Br J Dermatol 148:1222–1229PubMed

92.

Schmidt E, Seitz CS, Benoit S et al (2007) Rituximab in autoimmune bullous diseases: mixed responses and adverse effects. Br J Dermatol 156:352–356PubMed

93.

Schmidt E, Zillikens D (2011) The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Arztebl Int 108:399–405 (I–III)PubMedPubMedCentral

94.

Schmidt E, Zillikens D (2010) Immunoadsorption in dermatology. Arch Dermatol Res 302:241–253PubMed

95.

Schmidt E, Zillikens D (2010) Modern diagnosis of autoimmune blistering skin diseases. Autoimmun Rev 10:84–89PubMed

96.

Shimanovich I, Bohmke AK, Westermann L et al (2009) Successful treatment of severe pemphigus with the combination of immunoadsorption and rituximab. J Invest Dermatol 129:S97

97.

Shimanovich I, Herzog S, Schmidt E et al (2006) Improved protocol for treatment of pemphigus vulgaris with protein A immunoadsorption. Clin Exp Dermatol 31:768–774PubMed

98.

Spindler V, Rotzer V, Dehner C et al (2013) Peptide-mediated desmoglein 3 crosslinking prevents pemphigus vulgaris autoantibody-induced skin blistering. J Clin Invest 123:800–811PubMedPubMedCentral

99.

Spindler V, Vielmuth F, Schmidt E et al (2010) Protective endogenous cyclic adenosine 5′-monophosphate signaling triggered by pemphigus autoantibodies. J Immunol 185:6831–6838PubMedPubMedCentral

100.

Tavakolpour S (2017) Current and future treatment options for pemphigus: Is it time to move towards more effective treatments? Int Immunopharmacol 53:133–142PubMed

101.

Tony HP, Burmester G, Schulze-Koops H et al (2011) Safety and clinical outcomes of rituximab therapy in patients with different autoimmune diseases: experience from a national registry (GRAID). Arthritis Res Ther 13:R75PubMedPubMedCentral

102.

Toth GG, Van De Meer JB, Jonkman MF (2002) Dexamethasone pulse therapy in pemphigus. J Eur Acad Dermatol Venereol 16:607–611PubMed

103.

Tran KD, Wolverton JE, Soter NA (2013) Methotrexate in the treatment of pemphigus vulgaris: experience in 23 patients. Br J Dermatol 169:916–921PubMed

104.

Veldman C, Hohne A, Dieckmann D et al (2004) Type I regulatory T cells specific for desmoglein 3 are more frequently detected in healthy individuals than in patients with pemphigus vulgaris. J Immunol 172:6468–6475PubMed

105.

Vyas N, Patel NS, Cohen GF (2013) Mycophenolate mofetil as a first-line steroid-sparing agent in the treatment of pemphigus vulgaris. J Drugs Dermatol 12:210–216PubMed

106.

Wang HH, Liu CW, Li YC et al (2015) Efficacy of rituximab for pemphigus: a systematic review and meta-analysis of different regimens. Acta Derm Venereol 95:928–932PubMed

107.

Weng WK, Levy R (2003) Two immunoglobulin G fragment C receptor polymorphisms independently predict response to rituximab in patients with follicular lymphoma. J Clin Oncol 21:3940–3947PubMed

108.

Werth VP (1996) Treatment of pemphigus vulgaris with brief, high-dose intravenous glucocorticoids. Arch Dermatol 132:1435–1439PubMed

109.

Werth VP, Fivenson D, Pandya AG et al (2008) Multicenter randomized, double-blind, placebo-controlled, clinical trial of dapsone as a glucocorticoid-sparing agent in maintenance-phase pemphigus vulgaris. Arch Dermatol 144:25–32PubMed

110.

Whitworth JA (1987) Mechanisms of glucocorticoid-induced hypertension. Kidney Int 31:1213–1224PubMed

111.

Yang SK, Hong M, Baek J et al (2014) A common missense variant in NUDT15 confers susceptibility to thiopurine-induced leukopenia. Nat Genet 46:1017–1020PubMedPubMedCentral

112.

Zimmermann J, Bahmer F, Rose C et al (2010) Clinical and immunopathological spectrum of paraneoplastic pemphigus. J Dtsch Dermatol Ges 8:598–605PubMed

Titel: Aktuelle Therapie des Pemphigus
verfasst von: N. van Beek, MD
D. Zillikens
E. Schmidt
Publikationsdatum: 18.03.2019
Verlag: Springer Medizin
Schlagwörter: Pemphigus
Azathioprin
Rituximab
Glucocorticoide
CD20-Inhibitoren
Pemphigus vulgaris
Rituximab
Azathioprin
Mycophenolat-Mofetil
Prednisolon
Erschienen in: Die Dermatologie / Ausgabe 4/2019
Print ISSN: 2731-7005
Elektronische ISSN: 2731-7013
DOI: https://doi.org/10.1007/s00105-019-4385-9

Leitlinien kompakt für die Dermatologie

Mit medbee Pocketcards sicher entscheiden.

^{Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag}

Kostenlos registrieren

Neu im Fachgebiet Dermatologie

Studienlage spricht für Isotretinoin zur Rosazea-Therapie

23.05.2024 Rosazea Nachrichten

Isotretinoin wird off-label zur Behandlung von Rosazea eingesetzt. Wie solide die Evidenz dafür ist, wurde jetzt in einem systematischen Review überprüft.

So sicher sind Tattoos: Neue Daten zur Risikobewertung

22.05.2024 Melanom Nachrichten

Das größte medizinische Problem bei Tattoos bleiben allergische Reaktionen. Melanome werden dadurch offensichtlich nicht gefördert, die Farbpigmente könnten aber andere Tumoren begünstigen.

„Übersichtlicher Wegweiser“: Lauterbachs umstrittener Klinik-Atlas ist online

17.05.2024 Klinik aktuell Nachrichten

Sie sei „ethisch geboten“, meint Gesundheitsminister Karl Lauterbach: mehr Transparenz über die Qualität von Klinikbehandlungen. Um sie abzubilden, lässt er gegen den Widerstand vieler Länder einen virtuellen Klinik-Atlas freischalten.

Riesenzellarteriitis: 15% der Patienten sind von okkulter Form betroffen

16.05.2024 Riesenzellarteriitis Nachrichten

In einer retrospektiven Untersuchung haben Forschende aus Belgien und den Niederlanden die okkulte Form der Riesenzellarteriitis genauer unter die Lupe genommen. In puncto Therapie und Rezidivraten stellten sie keinen sehr großen Unterschied zu Erkrankten mit kranialen Symptomen fest.

Update Dermatologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.

Newsletter bestellen

Bildnachweise

Die Leitlinien für Ärztinnen und Ärzte, Nahaufnahme Rosazea auf der Wange/© Alessandro Grandini / stock.adobe.com (Symbolbild mit Fotomodell), Eine Tätowierung wird mit roter Farbe ausgefüllt/© nagaets / stock.adobe.com (Symbolbild mit Fotomodell), Operation/© Peakstock / Stock.adobe.com (Symbolbild mit Fotomodellen), Rechte Aa. carotis interna mit Wandverdickung bei Riesenzellarteriitis/© Kisyova H et al. | all rights reserved Springer Medizin Verlag GmbH

Live-Webinar "Urologie und Sexualmedizin in der Praxis"

Springer Medizin

Zusammenfassung

Hintergrund

Ziel der Arbeit

Methoden

Ergebnisse

Diskussion

Bitte loggen Sie sich ein, um Zugang zu diesem Inhalt zu erhalten

Weitere Artikel der Ausgabe 4/2019

Epidermolysis bullosa acquisita

Ein 7‑jähriger Junge mit leicht juckenden, weißlichen, stecknadelkopfgroßen Papeln

Dermatologische Tumornachsorge bei Retinoblastompatienten

Bullöse Autoimmundermatosen im Kindesalter

Panorama Dermatologische Praxis