Pemphigus

Pemphigus vulgaris and paraneoplastic pemphigus are 2 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 cutane...

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Bibliographic Details
Published inThe Dental clinics of North America Vol. 57; no. 4; p. 597
Main Authors Santoro, Frank A, Stoopler, Eric T, Werth, Victoria P
Format Journal Article
LanguageEnglish
Published United States 01.10.2013
Subjects
Adrenal Cortex Hormones - therapeutic use
Antibodies, Monoclonal - therapeutic use
Diagnosis, Differential
Humans
Mouth Mucosa - pathology
Mouth Neoplasms - diagnosis
Pemphigus - classification
Pemphigus - drug therapy
Pemphigus - pathology
Severity of Illness Index
Stomatitis
Blistering disorder
Paraneoplastic pemphigus
Oral erosions
Pemphigus
Acantholysis
Rituximab
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Summary:Pemphigus vulgaris and paraneoplastic pemphigus are 2 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. 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.
ISSN:1558-0512
DOI:10.1016/j.cden.2013.06.002