ORAL MYTH SERIES

Urban legends: pemphigus vulgaris

  1. N Cirillo1,
  2. E Cozzani2,
  3. M Carrozzo3,
  4. SA Grando4

Article first published online: 15 FEB 2012

DOI: 10.1111/j.1601-0825.2011.01899.x

Additional Information

How to Cite

Cirillo, N., Cozzani, E., Carrozzo, M. and Grando, S. (2012), Urban legends: pemphigus vulgaris. Oral Diseases. doi: 10.1111/j.1601-0825.2011.01899.x

Author Information

  1. 1

    Department of Oral and Dental Sciences, University of Bristol, Bristol, UK

  2. 2

    DISSAL, Section of Dermatology, University of Genoa, Genoa, Italy

  3. 3

    Centre for Oral Health Research, Oral Medicine Department, Newcastle University, UK

  4. 4

    Department of Dermatology, University of California, Irvine, CA, USA

*Nicola Cirillo, School of Oral and Dental Sciences, University of Bristol, Lower Maudlin street, Bristol BS1 2LY, UK. Tel: +44 (0)117 3423453, Fax: +44 (0)117 3424428, E-mail: n.cirillo@bristol.ac.uk

Publication History

  1. Article first published online: 15 FEB 2012
  2. Accepted manuscript online: 23 DEC 2011 11:54AM EST
  3. Received: 27 October 2011; revised 5 December 2011; accepted 12 December 2011

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Keywords:

  • pemphigus;
  • acantholysis;
  • desmogleins;
  • apoptosis;
  • rituximab

Oral Diseases (2012) doi: 10.1111/j.1601-0825.2012.01899.x

Pemphigus vulgaris (PV) is the most common type of pemphigus. PV pathogenesis is still debated, and treatment remains challenging. We investigated five controversial topics: (1) What are the target antigens in PV? (2) Do desmogleins adequately address PV pathophysiology? (3) How does acantholysis occur in PV? (4) Is PV still a lethal disease? (5) What is the role of rituximab (RTX) in PV treatment? Results from extensive literature searches suggested the following: (1) Target antigens of PV include a variety of molecules and receptors that are not physically compartmentalized within the epidermis. (2) PV is caused by a variety of autoantibodies to keratinocyte self-antigens, which concur to cause blistering by acting synergistically. (3) The concept of apoptolysis distinguishes the unique mechanism of autoantibody-induced keratinocyte damage in PV from other known forms of cell death. (4) PV remains potentially life-threatening largely because of treatment side effects, but it is uncertain which therapies carry the highest likelihood of lethal risk. (5) RTX is a very promising treatment option in patients with widespread recalcitrant or life-threatening PV. RTX’s cost is an issue, its long-term side effects are still unknown, and randomized controlled trials are needed to establish the optimal dosing regimen.

View Full Article with Supporting Information (HTML) Get PDF (654K)