Pemphigus Foliaceus

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


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.


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



Autoimmune bullous disorders


Complete remission off therapy


Complete remission on therapy


Dexamethasone and cyclophosphamide pulse therapy


Direct immunofluorescence




Enzyme-linked immunosorbent assay


Glucose-6-phosphate dehydrogenase


Indirect immunofluorescence


Intravenous immunoglobulin


Lupus erythematosus


Mycophenolate mofetil




Pemphigus erythematosus


Pemphigus foliaceus


Pemphigus herpetiformis


Partial remission off therapy


Partial remission on minimal therapy


Pemphigus vulgaris




Thiopurine methyl transferase


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© 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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