title: Pemphigus.
abstract: 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.
authors.affiliation: Department of Dermatology, Henry Ford Hospital, 3031 West Grand Boulevard, Suite 800, Detroit, MI 48202, USA; Department of Dermatology, Philadelphia VA Medical Center, 3900 Woodland Avenue, Philadelphia, PA 19104, USA.
authors.forename: Frank A
authors.initials: FA
authors.lastname: Santoro
authors.forename: Eric T
authors.initials: ET
authors.lastname: Stoopler
authors.forename: Victoria P
authors.initials: VP
authors.lastname: Werth
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D006801:Humans; D009061:Mouth Mucosa / Q000473:pathology*; D009062:Mouth Neoplasms / Q000175:diagnosis*; D010392:Pemphigus / Q000145:classification / Q000188:drug therapy / Q000473:pathology; D012720:Severity of Illness Index
chemical_list: D000305:Adrenal Cortex Hormones; D000911:Antibodies, Monoclonal
keywords: Acantholysis; Blistering disorder; Oral erosions; Paraneoplastic pemphigus; Pemphigus; Rituximab; Stomatitis
references.citation: J Am Acad Dermatol. 1999 Feb;40(2 Pt 1):167-70
references.pmid: 10025740
references.citation: Osteoporos Int. 2007 Oct;18(10):1319-28
references.pmid: 17566815
references.citation: Expert Rev Clin Immunol. 2011 Jul;7(4):529-41
references.pmid: 21790294
references.citation: Dermatologica. 1978;157(3):138-45
references.pmid: 680299
references.citation: Int J Dermatol. 2011 Aug;50(8):905-14
references.pmid: 21781058
references.citation: J Am Acad Dermatol. 1995 Mar;32(3):441-7
references.pmid: 7868713
references.citation: Br J Dermatol. 1993 Oct;129(4):461-4
references.pmid: 8217764
references.citation: J Am Acad Dermatol. 2001 Dec;45(6):803-22; quiz 822-4
references.pmid: 11712024
references.citation: Oral Maxillofac Surg Clin North Am. 2008 Nov;20(4):577-84
references.pmid: 18940624
references.citation: J Am Acad Dermatol. 1989 Oct;21(4 Pt 1):677-80
references.pmid: 2808784
references.citation: Br J Oral Maxillofac Surg. 2008 Jun;46(4):272-7
references.pmid: 17870218
references.citation: Eur J Dermatol. 2003 Mar-Apr;13(2):142-4
references.pmid: 12695129
references.citation: Int J Dermatol. 2005 Jul;44(7):541-4
references.pmid: 15985020
references.citation: Br J Dermatol. 2001 Jun;144(6):1102-4
references.pmid: 11422027
references.citation: J Eur Acad Dermatol Venereol. 2013 Apr;27(4):430-5
references.pmid: 22221266
references.citation: J Am Acad Dermatol. 2008 Jun;58(6):1043-6
references.pmid: 18339444
references.citation: Br Med J (Clin Res Ed). 1983 Dec 10;287(6407):1761-2
references.pmid: 6416581
references.citation: J Invest Dermatol. 1998 Jan;110(1):76-8
references.pmid: 9424092
references.citation: J Can Dent Assoc. 2005 Oct;71(9):667-72
references.pmid: 16271165
references.citation: Int J Dermatol. 2011 Sep;50(9):1120-3
references.pmid: 22126875
references.citation: J Oral Sci. 2010 Sep;52(3):449-54
references.pmid: 20881339
references.citation: Compend Contin Educ Dent. 2010 Jun;31(5):344-50, 352-9; quiz 362, 364
references.pmid: 20583504
references.citation: N Engl J Med. 1990 Dec 20;323(25):1729-35
references.pmid: 2247105
references.citation: Dermatol Ther. 2012 Jan-Feb;25(1):95-7
references.pmid: 22591504
references.citation: J Invest Dermatol. 2009 Oct;129(10):2404-10
references.pmid: 19357707
references.citation: Dermatology. 2003;206(4):351-2
references.pmid: 12771484
references.citation: Dent Clin North Am. 2005 Jan;49(1):107-25, viii-ix
references.pmid: 15567364
references.citation: J Eur Acad Dermatol Venereol. 2008 Sep;22(9):1070-5
references.pmid: 18410336
references.citation: Br J Dermatol. 2012 Apr;166(4):844-52
references.pmid: 22092243
references.citation: Cancer. 1974 Apr;33(4):1091-9
references.pmid: 4362104
references.citation: Arch Dermatol. 2012 Sep;148(9):1031-6
references.pmid: 22710375
references.citation: J Cell Biol. 1993 Jul;122(2):409-15
references.pmid: 8320263
references.citation: Dermatol Clin. 2011 Jul;29(3):393-404, vii-iii
references.pmid: 21605804
references.citation: J Investig Dermatol Symp Proc. 2004 Jan;9(1):29-33
references.pmid: 14870982
references.citation: Oral Dis. 2008 Oct;14(7):640-3
references.pmid: 18627505
references.citation: Lancet. 1999 Aug 21;354(9179):667-72
references.pmid: 10466686
references.citation: Br J Dermatol. 2001 Apr;144(4):775-80
references.pmid: 11298536
references.citation: J Invest Dermatol. 2012 Jul;132(7):1798-805
references.pmid: 22437316
references.citation: Oral Surg Oral Med Oral Pathol. 1980 Sep;50(3):244-9
references.pmid: 6931999