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Clinical and immunological features of pemphigus relapse.

Authors
  • Ujiie, I1
  • Ujiie, H1
  • Iwata, H1
  • Shimizu, H1
  • 1 Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan. , (Japan)
Type
Published Article
Journal
British Journal of Dermatology
Publisher
Wiley (Blackwell Publishing)
Publication Date
Jun 01, 2019
Volume
180
Issue
6
Pages
1498–1505
Identifiers
DOI: 10.1111/bjd.17591
PMID: 30585310
Source
Medline
Language
English
License
Unknown

Abstract

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. To elucidate the risk factors and clinical features of pemphigus relapse. We carried out a retrospective review of the clinical records of 42 cases of pemphigus at a single centre. Sixty-two per cent of patients experienced relapse, usually when oral prednisolone was tapered to around 0·1 mg kg-1 . In mucocutaneous pemphigus vulgaris (mcPV), the initial doses (mean ± SD) of prednisolone were significantly lower in patients with relapse (0·78 ± 0·24 mg kg-1 ) than in those without relapse (1·01 ± 0·01 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%). 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 0·1 mg kg-1 may lead to better outcomes. © 2018 British Association of Dermatologists.

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