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Pemphigus Foliaceus

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Autoimmune Bullous Diseases

Abstract

Pemphigus foliaceus (PF) is a group of autoimmune skin blistering diseases comprising four subtypes: Cazenave, endemic, erythematosus, and herpetiformis. The common characteristic is the frequent presence of pathogenic anti-desmoglein 1 antibodies leading to bullae in the skin. Other characteristics – clinical, histological, pathogenesis, and therapy – vary and are specified in this chapter.

Pemphigus foliaceus Cazenave first described by Cazenave is the classical form of PF. The elemental dermatological lesions are erythematous plaques with pastry puff squames predominantly affecting seborrheic areas. The histopathological hallmark is acantholysis in the upper part of the epidermis (subcorneal or intraspinous). The disease is caused by autoantibodies to desmoglein 1 on the epithelial cell surface. Therapy is based on systemic corticosteroids and immunosuppressive adjuvants. Rituximab and other anti-CD20 biologics targeting B lymphocytes have changed the prognosis of the disease from chronic relapsing to long-term remissions.

Endemic pemphigus foliaceus is a variant of PF that is endemic under peasants in rural Brazil, Indian tribes and gold miners in Colombia, and under women in Tunisia. The histopathology, immunology, and therapy are similar to PF Cazenave.

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Further Reading

  • Hertl M, Jedlickova H, Karpati S, Marinovic B, Uzun S, Yayli S, Mimouni D, Borradori L, Feliciani C, Ioannides D, Joly P, Kowalewski C, Zambruno G, Zillikens D, Jonkman MF. Pemphigus. S2 guideline for diagnosis and treatment - guided by the European Dermatology Forum (EDF) in cooperation with the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2014;29:405–14.

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Authors and Affiliations

  1. Department of Dermatology, Paulista School of Medicine, Federal University of São Paulo, Hospital São Paulo, São Paulo, Brazil

    Laura de Sena Nogueira Maehara MD

  2. Department of Dermatology, Center for Blistering Diseases, University Medical Center Groningen, University of Groningen, Groningen, the Netherlands

    Laura de Sena Nogueira Maehara MD & Marcel F. Jonkman MD, PhD

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  1. Laura de Sena Nogueira Maehara MD
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  2. Marcel F. Jonkman MD, PhD
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Correspondence to Laura de Sena Nogueira Maehara MD .

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Editors and Affiliations

  1. Department of Dermatology, University Medical Center Groningen Center for Blistering Diseases, Groningen, The Netherlands

    Marcel F. Jonkman

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© 2016 Springer International Publishing Switzerland

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Maehara, L.d.S.N., Jonkman, M.F. (2016). Pemphigus Foliaceus. In: Jonkman, M. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-23754-1_9

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  • DOI: https://doi.org/10.1007/978-3-319-23754-1_9

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-23753-4

  • Online ISBN: 978-3-319-23754-1

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