Title

Pemphigus

Document Type

Journal Article

Date of this Version

10-2013

Publication Source

Dental Clinics of North America

Volume

57

Issue

4

Start Page

597

Last Page

610

DOI

10.1016/j.cden.2013.06.002

Abstract

Pemphigus vulgaris and paraneoplastic pemphigus are two subtypes of pemphigus that involve the oral mucosa. These autoimmune blistering disorders have antibodies targeted against proteins of keratinocyte adhesion, thereby causing acantholysis. Clinical findings include oral erosions and flaccid cutaneous bullae and erosions. In addition to the clinical exam, diagnostic tests including tissue biopsy, direct and indirect immunofluorescence, and enzyme-linked immunosorbent assays (ELISA) help to establish a diagnosis. Further malignancy workup in patients with suspected paraneoplastic pemphigus is warranted. Retrospective uncontrolled studies suggest that immunosuppressive agents reduce mortality in pemphigus vulgaris and cohort uncontrolled studies of rituximab, a monoclonal antibody against CD20, suggest it is an effective treatment for refractory patients. Ongoing studies will define its role in early disease.

Copyright/Permission Statement

© 2013. This manuscript version is made available under the CC-BY-NC-ND 4.0 license http://creativecommons.org/licenses/by-nc-nd/4.0/

Keywords

pemphigus, paraneoplastic pemphigus, oral erosions, stomatitis, blistering disorder, acantholysis, rituximab

 

Date Posted: 30 June 2015