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Urban legends: pemphigus vulgaris

Lookup NU author(s): Professor Marco CarrozzoORCiD

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Abstract

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.

Publication metadata

Author(s): Cirillo N, Cozzani E, Carrozzo M, Grando SA

Publication type: Article

Publication status: Published

Journal: Oral Diseases

Year: 2012

Volume: 18

Issue: 5

Pages: 442-458

Print publication date: 15/02/2012

ISSN (print): 1354-523X

ISSN (electronic): 1601-0825

Publisher: Wiley-Blackwell Publishing, Inc.

URL: http://dx.doi.org/10.1111/j.1601-0825.2011.01899.x

DOI: 10.1111/j.1601-0825.2011.01899.x

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