Background More than half of patients with pemphigus experience relapse during the disease course. The risk factors and clinical and immunological characteristics of relapse remain largely unclear. Objectives To elucidate the risk factors and clinical features of pemphigus relapse. Methods We carried out a retrospective review of the clinical records of 42 cases of pemphigus at a single centre. Results Sixty-two per cent of patients experienced relapse, usually when oral prednisolone was tapered to around 0 center dot 1 mg kg(-1). In mucocutaneous pemphigus vulgaris (mcPV), the initial doses (mean +/- SD) of prednisolone were significantly lower in patients with relapse (0 center dot 78 +/- 0 center dot 24 mg kg(-1)) than in those without relapse (1 center dot 01 +/- 0 center dot 01 mg kg(-1)). At relapse, mcPV shifted to mucosal dominant PV (mPV; 40%), pemphigus foliaceus (PF) (20%) or 'other' (20%). In contrast, relapsing mPV and PF had the same clinical phenotypes as the initial phenotypes. Patients with both anti-desmoglein (Dsg)1 and anti-Dsg3 antibodies at onset had recurrence with anti-Dsg3 antibodies alone (40%), with both anti-Dsg1 and anti-Dsg3 antibodies (30%), with anti-Dsg1 antibody alone (20%) or were subthreshold (10%). Conclusions mcPV shows transitions in clinical phenotype and autoantibody profile at relapse. At least 1 mg kg(-1) daily of prednisolone, especially for patients with mcPV, and prudent tapering around 0 center dot 1 mg kg(-1) may lead to better outcomes.
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Med Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Weiss, Doris
Ristl, Robin
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Med Univ Vienna, Ctr Med Stat Informat & Intelligent Syst, Sect Med Stat, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Ristl, Robin
Griss, Johannes
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Med Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Griss, Johannes
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Bangert, Christine
Foedinger, Dagmar
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Med Univ Vienna, Dept Dermatol, Div Gen Dermatol, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Foedinger, Dagmar
Stingl, Georg
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Med Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Stingl, Georg
Brunner, Patrick M.
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Med Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
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Univ Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, BrazilUniv Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
Machado Schroeder, Franciane Maria
Palma, Victor de Mello
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Univ Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, BrazilUniv Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
Palma, Victor de Mello
Rados, Pantelis Varvaki
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Univ Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, BrazilUniv Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
Rados, Pantelis Varvaki
Visioli, Fernanda
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Univ Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
Hosp Clin Porto Alegre, Expt Ctr Res, Porto Alegre, RS, BrazilUniv Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
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Med Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Weiss, Doris
Ristl, Robin
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h-index: 0
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Med Univ Vienna, Ctr Med Stat Informat & Intelligent Syst, Sect Med Stat, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Ristl, Robin
Griss, Johannes
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Med Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Griss, Johannes
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机构:
Bangert, Christine
Foedinger, Dagmar
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h-index: 0
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Med Univ Vienna, Dept Dermatol, Div Gen Dermatol, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Foedinger, Dagmar
Stingl, Georg
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h-index: 0
机构:
Med Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
Stingl, Georg
Brunner, Patrick M.
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h-index: 0
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Med Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, AustriaMed Univ Vienna, Dept Dermatol, Div Immunol Allergy & Infect Dis, A-1090 Vienna, Austria
机构:
Univ Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, BrazilUniv Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
Machado Schroeder, Franciane Maria
Palma, Victor de Mello
论文数: 0引用数: 0
h-index: 0
机构:
Univ Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, BrazilUniv Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
Palma, Victor de Mello
Rados, Pantelis Varvaki
论文数: 0引用数: 0
h-index: 0
机构:
Univ Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, BrazilUniv Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
Rados, Pantelis Varvaki
Visioli, Fernanda
论文数: 0引用数: 0
h-index: 0
机构:
Univ Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil
Hosp Clin Porto Alegre, Expt Ctr Res, Porto Alegre, RS, BrazilUniv Fed Rio Grande do Sul, Sch Dent, Oral Med Dept, Porto Alegre, RS, Brazil