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Pemphigus Foliaceus

  • Kara HeelanEmail author
  • Scott Walsh
  • Neil H. Shear
Chapter

Abstract

Pemphigus foliaceus is a rare autoimmune disease that results in blistering of the skin. It is caused by autoantibodies directed against cell-surface antigens on keratinocytes, which when targeted lose their cellular adhesion properties and separate from one another to form blisters within the epidermis. The disease has two predominant types: endemic and sporadic. In pemphigus foliaceus the blisters are high in the epidermis, just below the stratum corneum, and are associated with antibodies against desmoglein-1. The disease is diagnosed based on its clinical manifestations (flaccid blisters and erosions on skin), histology (epidermal acantholysis), and immunological abnormalities (circulating and tissue-fixed antibodies against keratinocyte surface antigens). This chapter summarizes the epidemiology, clinical features and diagnostic techniques. An in-depth review of treatment modalities reported in the literature is presented and includes topical agents, anti-inflammatory agents, immunosuppressant and biologic therapy. We also present a treatment approach based on the authors’ experience of treating this rare disease.

Keywords

Pemphigus foliaceus Dapsone Methotrexate Azathioprine Mycophenolate mofetil Intravenous immunoglobulin Cyclophosphamide Plasmapheresis Rituximab Plaquenil Colchicine Tetracyclines Treatment algorithm for pemphigus foliaceus 

Abbreviations

AIBD

Autoimmune bullous disorders

CR

Complete remission off therapy

CROT

Complete remission on therapy

DCP

Dexamethasone and cyclophosphamide pulse therapy

DIF

Direct immunofluorescence

dsg

Desmoglein

ELISA

Enzyme-linked immunosorbent assay

G-6PD

Glucose-6-phosphate dehydrogenase

IIF

Indirect immunofluorescence

IVIg

Intravenous immunoglobulin

LE

Lupus erythematosus

MMF

Mycophenolate mofetil

MTX

Methotrexate

PE

Pemphigus erythematosus

PF

Pemphigus foliaceus

PH

Pemphigus herpetiformis

PR

Partial remission off therapy

PROT

Partial remission on minimal therapy

PV

Pemphigus vulgaris

RTX

Rituximab

TPMT

Thiopurine methyl transferase

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Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  1. 1.Department of DermatologyUniversity College London Hospital (UCLH)LondonUK
  2. 2.Division of Dermatology, Department of MedicineSunnybrook Health Sciences Centre/University of TorontoTorontoCanada
  3. 3.Division of Dermatology, Department of Medicine (Clinical Pharmacology)Sunnybrook Health Sciences Centre/University of TorontoTorontoCanada

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