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Pemphigus Foliaceus and Pemphigus Erythematosus

  • Christoph M. Hammers
  • Enno SchmidtEmail author
  • Luca Borradori
Chapter

Abstract

Pemphigus foliaceus (PF) is a chronic autoimmune blistering disease of the skin, characterized by autoantibodies against a structural component of the epidermal desmosome, desmoglein 1 (Dsg1). Pemphigus erythematosus (PE; syn. Senear-Usher syndrome) is a localized form of PF with lesions typically developing on the face and the upper trunk and back. Management of pemphigus foliaceus does not greatly differ from that of pemphigus vulgaris. Systemic corticosteroids represent the mainstay of therapy and are usually combined with an immunosuppressive adjuvant.

Keywords

Desmoglein 1 Fogo selvagem Review Senear-Usher syndrome Treatment 

Abbreviations

Abs

Antibodies

ACR

American College of Rheumatology

AZA

Azathioprine

CS

Corticosteroids

CYC

Cyclophosphamide

Dsg

Desmoglein

IA

Immunoadsorption

IF

Immunofluorescence

IVIg

Intravenous immunoglobulins

MMF

Mycophenolate mofetil

MPA

Mycophenolic acid

MTX

Methotrexate

PE

Pemphigus erythematosus

PF

Pemphigus foliaceus

RCT

Randomized controlled clinical trials

RTX

Rituximab

TPMT

Thiopurine methyltransferase

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  • Christoph M. Hammers
    • 1
    • 2
  • Enno Schmidt
    • 2
    Email author
  • Luca Borradori
    • 3
  1. 1.Department of DermatologyUniversity of PennsylvaniaPhiladelphiaUSA
  2. 2.Department of DermatologyUniversity of LübeckLübeckGermany
  3. 3.Department of DermatologyBern University HospitalBernSwitzerland

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