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Autoimmune Bullous Skin Disorders

  • Michael Hertl
  • Emmanuel Laffitte
  • Luca Borradori
  • Christian Rose
  • Detlef Zillikens
  • Mei Chen
  • Dafna Hallel-Halevy
  • Celina Nadelman
  • David T. Woodley

Abstract

Pemphigus (from the Greek pemphix, meaning bubble or blister) encompasses a group of life-threatening autoimmune blistering diseases characterized by intraepithelial blister formation (Lever 1953; Huilgol et al. 1995; Hertl 2000). The molecular basis for blister formation is a loss of adhesion between keratinocytes caused by circulating autoantibodies (auto-Ab) directed against intercellular adhesion structures of epidermal keratinocytes (Amagai et al. 1991; Bedane et al. 1996). Several forms of pemphigus have been classified depending on the level of the intraepidermal split formation. In the pemphigus vulgaris (PV) group, the blisters are located just above the basal layer whereas in the pemphigus foliaceus (PF) group, the blisters occur within the upper layers of the epidermis (Bedane et al. 1996). Other members of the pemphigus group are paraneoplastic pemphigus (PNP), which generally occurs in patients with lymphoma, and drug-induced pemphigus, which usually develops after the administration of penicillamine. Based on recent immunological studies, a molecular distinction can be made by the characterization of the specificity of the auto-Ab that recognize different molecular target structures.

Keywords

Human Leukocyte Antigen Class Bullous Pemphigoid Dermatitis Herpetiformis Basement Membrane Zone Pemphigus Foliaceus 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Wien 2001

Authors and Affiliations

  • Michael Hertl
  • Emmanuel Laffitte
  • Luca Borradori
  • Christian Rose
  • Detlef Zillikens
  • Mei Chen
  • Dafna Hallel-Halevy
  • Celina Nadelman
  • David T. Woodley

There are no affiliations available

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