Advertisement

Pemphigus Foliaceus

  • Laura de Sena Nogueira MaeharaEmail author
  • Marcel F. Jonkman
Chapter

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.

Keywords

Desmosome Immunoglobulin Autoimmune disease Vesiculobullous disease Pemphigus 

References

  1. 1.
    Jonkman MF. JAMA dermatology patient page pemphigus. JAMA Dermatol. 2014;150:680.CrossRefPubMedGoogle Scholar
  2. 2.
    Amagai M. Desmoglein as a target in autoimmunity and infection. J Am Acad Dermatol. 2003;48:244–52.CrossRefPubMedGoogle Scholar
  3. 3.
    Abreu-Velez AM, Reason IJ, Howard MS, Roselino AM. Endemic pemphigus foliaceus over a century: part I. N Am J Med Sci. 2010;2:51–9.PubMedPubMedCentralGoogle Scholar
  4. 4.
    Bastuji-Garin S, Turki H, Mokhtar I, Nouira R, Fazaa B, Jomaa B, Zahaf A, Osman AB, Souissi R, Hémon D, Roujeau JC, Kamoun MR. Possible relation of Tunisian pemphigus with traditional cosmetics: a multicenter case–control study. Am J Epidemiol. 2002;155:249–56.CrossRefPubMedGoogle Scholar
  5. 5.
    de Waard M, Jonkman MF. Bullous diseases. In: Hamerlinck F, Lambert J, Neumann H, editors. Textbook of ethnic dermatology Haarlem. the Netherlands: DCHG medical communication; 2012. p. 157.Google Scholar
  6. 6.
    Lombardi C, Borges PC, Chaul A, Sampaio SA, Rivitti EA, Friedman H, Martins CR, Sanches Júnior JA, Cunha PR, Hoffmann RG, Diaz LA. Environmental risk factors in endemic pemphigus foliaceus (fogo selvagem). “The cooperative group on fogo selvagem research”. J Invest Dermatol. 1992;98:847–50.CrossRefPubMedGoogle Scholar
  7. 7.
    Goncalves GA, Brito MM, Salathiel AM, Ferraz TS, Alves D, Roselino AM. Incidence of pemphigus vulgaris exceeds that of pemphigus foliaceus in a region where pemphigus foliaceus is endemic: analysis of a 21-year historical series. An Bras Dermatol. 2011;86:1109–12.CrossRefPubMedGoogle Scholar
  8. 8.
    Jablonska S, Chorzelski TP, Beutner EH, Chorzelska J. Herpetiform pemphigus, a variable pattern of pemphigus. Int J Dermatol. 1975;14:353–9.CrossRefPubMedGoogle Scholar
  9. 9.
    Santi CG, Maruta CW, Aoki V, Sotto MN, Rivitti EA, Diaz LA. Pemphigus herpetiformis is a rare clinical expression of nonendemic pemphigus foliaceus, fogo selvagem, and pemphigus vulgaris. Cooperative Group on Fogo Selvagem Research. J Am Acad Dermatol. 1996;34:40–6.CrossRefPubMedGoogle Scholar
  10. 10.
    Kasperkiewicz M, Kowalewski C, Jabłońska S. Pemphigus herpetiformis: from first description until now. J Am Acad Dermatol. 2014;70:780–7.CrossRefPubMedGoogle Scholar
  11. 11.
    Senear FE, Usher B. An unusual type of pemphigus combining features of lupus erythematosus. Arch Dermatol. 1926;13:761–81.Google Scholar
  12. 12.
    Chorzelski T, Jablońska S, Blaszczyk M. Immunopathological investigations in the Senear-Usher syndrome (coexistence of pemphigus and lupus erythematosus). Br J Dermatol. 1968;80:211–7.CrossRefPubMedGoogle Scholar
  13. 13.
    Oktarina DAM, Poot AM, Kramer D, Diercks GFH, Jonkman MF, Pas HH. The IgG “lupus-band” deposition pattern of pemphigus erythematosus: association with the desmoglein 1 ectodomain as revealed by 3 cases. Arch Dermatol. 2012;148:1173–8.CrossRefPubMedGoogle Scholar

Further Reading

  1. 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.CrossRefPubMedGoogle Scholar

On the Web

  1. International Pemphigus & Pemphigoid Foundation http://www.pemphigus.org/.
  2. JAMA Dermatology Patient Page, Pemphigus http://archderm.jamanetwork.com/article.aspx?articleid=1879985.

Copyright information

© Springer International Publishing Switzerland 2016

Authors and Affiliations

  • Laura de Sena Nogueira Maehara
    • 1
    • 2
    Email author
  • Marcel F. Jonkman
    • 2
  1. 1.Department of DermatologyPaulista School of Medicine, Federal University of São Paulo, Hospital São PauloSão PauloBrazil
  2. 2.Department of DermatologyCenter for Blistering Diseases, University Medical Center Groningen, University of GroningenGroningenthe Netherlands

Personalised recommendations