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Pemphigus Foliaceus

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Autoimmune Bullous Diseases

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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.

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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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Authors and Affiliations

  1. Department of Dermatology, University College London Hospital (UCLH), 250 Euston Road, London, NW1 2PG, UK

    Kara Heelan MB BCH, BAO, MRCPI

  2. Division of Dermatology, Department of Medicine, Sunnybrook Health Sciences Centre/University of Toronto, Toronto, ON, Canada

    Scott Walsh MD, PhD

  3. Division of Dermatology, Department of Medicine (Clinical Pharmacology), Sunnybrook Health Sciences Centre/University of Toronto, Toronto, ON, Canada

    Neil H. Shear MD, FRCPC

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  1. Kara Heelan MB BCH, BAO, MRCPI
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  2. Scott Walsh MD, PhD
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  3. Neil H. Shear MD, FRCPC
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Correspondence to Kara Heelan MB BCH, BAO, MRCPI .

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Editors and Affiliations

  1. Department of Dermatology, University of Alabama at Birmingham, BIRMINGHAM, Alabama, USA

    Naveed Sami

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Heelan, K., Walsh, S., Shear, N.H. (2016). Pemphigus Foliaceus. In: Sami, N. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-26728-9_2

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