Highlights & Basics
- Pemphigus encompasses a group of potentially life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin.
- Pemphigus vulgaris (PV) is the most common variant and affects skin and mucosa.
- Pemphigus foliaceus (PF) is confined to the skin. Paraneoplastic pemphigus (PNP) is rare; it can involve the skin and mucosal surfaces of the eyes, mouth, nasopharynx, and esophagus.
- Diagnosis is based on clinical findings of erosions (epidermal loss) and blisters of the skin, mucosa, or both.
- Direct immunofluorescence staining of biopsied skin shows immunoglobulin and C3 deposits on the keratinocyte surface, and histology reveals keratinocyte acantholysis.
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History & Exam
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Definition
Epidemiology
Etiology
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Citations
Joly P, Horvath B, Patsatsi alpha, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV). J Eur Acad Dermatol Venereol. 2020 Sep;34(9):1900-13.[Abstract][Full Text]
Kasperkiewicz M, Ellebrecht CT, Takahashi H, et al. Pemphigus. Nat Rev Dis Primers. 2017 May 11;3:17026.[Abstract]
Murrell DF, Peña S, Joly P, et al. Diagnosis and management of pemphigus: recommendations of an international panel of experts. J Am Acad Dermatol. 2020 Mar;82(3):575-85.e1.[Abstract]
Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017 May 20;389(10083):2031-40.[Abstract]
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5. Kasperkiewicz M, Ellebrecht CT, Takahashi H, et al. Pemphigus. Nat Rev Dis Primers. 2017 May 11;3:17026.[Abstract]
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8. Alpsoy E, Akman-Karakas A, Uzun S. Geographic variations in epidemiology of two autoimmune bullous diseases: pemphigus and bullous pemphigoid. Arch Dermatol Res. 2015 May;307(4):291-8.[Abstract][Full Text]
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11. Ohzono A, Sogame R, Li X, et al. Clinical and immunological findings in 104 cases of paraneoplastic pemphigus. Br J Dermatol. 2015 Dec;173(6):1447-52.[Abstract]
12. Leger S, Picard D, Ingen-Housz-Oro S, et al. Prognostic factors of paraneoplastic pemphigus. Arch Dermatol. 2012 Oct;148(10):1165-72.[Abstract][Full Text]
13. Becker BA, Gaspari AA. Pemphigus vulgaris and vegetans. Dermatol Clin. 1993 Jul;11(3):429-52.[Abstract]
14. Crosby DL, Diaz LA. Endemic pemphigus foliaceus. Fogo selvagem. Dermatol Clin. 1993 Jul;11(3):453-62.[Abstract]
15. Anhalt GJ, Kim SC, Stanley JR, et al. Paraneoplastic pemphigus - an autoimmune mucocutaneous disease associated with neoplasia. N Engl J Med. 1990 Dec 20;323(25):1729-35.[Abstract][Full Text]
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17. Vernal S, Pepinelli M, Casanova C, et al. Insights into the epidemiological link between biting flies and pemphigus foliaceus in southeastern Brazil. Acta Trop. 2017 Dec;176:455-62.[Abstract]
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20. Peng B, Temple BR, Yang J, et al. Identification of a primary antigenic target of epitope spreading in endemic pemphigus foliaceus. J Autoimmun. 2021 Jan;116:102561.[Abstract]
21. Harman KE, Gratian MJ, Bhogal BS, et al. A study of desmoglein 1 autoantibodies in pemphigus vulgaris: racial differences in frequency and the association with a more severe phenotype. Br J Dermatol. 2000 Aug;143(2):343-8.[Abstract]
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23. Billet SE, Grando SA, Pittelkow MR. Paraneoplastic autoimmune multiorgan syndrome: review of the literature and support for a cytotoxic role in pathogenesis. Autoimmunity. 2006 Nov;39(7):617-30.[Abstract]
24. Bordenave K, Griffith J, Hordes SM, et al. The historical and geomedical immunogenetics of pemphigus among the descendants of Sephardic Jews in New Mexico. Arch Dermatol. 2001 Jun;137(6):825-6.[Abstract]
25. Brenner S, Goldberg I. Drug-induced pemphigus. Clin Dermatol. Jul-Aug 2011;29(4):455-7.[Abstract]
26. Murrell DF, Peña S, Joly P, et al. Diagnosis and management of pemphigus: recommendations of an international panel of experts. J Am Acad Dermatol. 2020 Mar;82(3):575-85.e1.[Abstract]
27. Raneses E, McGinley Simpson M, Rosenberg A, et al. A retrospective review of autoimmune bullous disease antibody positivity before clinical symptoms. J Am Acad Dermatol. 2021 Feb 4;S0190-9622(21)00315-7.[Abstract]
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29. Nousari HC, Deterding R, Wojtczack H, et al. The mechanism of respiratory failure in paraneoplastic pemphigus. N Engl J Med. 1999 May 6;340(18):1406-10.[Abstract]
30. Cummins DL, Mimouni D, Tzu J, et al. Lichenoid paraneoplastic pemphigus in the absence of detectable antibodies. J Am Acad Dermatol. 2007 Jan;56(1):153-9.[Abstract]
31. Joly P, Maho-Vaillant M, Prost-Squarcioni C, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet. 2017 May 20;389(10083):2031-40.[Abstract]
32. Wang HH, Liu CW, Li YC, et al. Efficacy of rituximab for pemphigus: a systematic review and meta-analysis of different regimens. Acta Derm Venereol. 2015 Nov;95(8):928-32.[Abstract][Full Text]
33. Tavakolpour S, Mahmoudi H, Balighi K, et al. Sixteen-year history of rituximab therapy for 1085 pemphigus vulgaris patients: A systematic review. Int Immunopharmacol. 2018 Jan;54:131-8.[Abstract]
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35. Beissert S, Mimouni D, Kanwar AJ, et al. Treating pemphigus vulgaris with prednisone and mycophenolate mofetil: a multicenter, randomized, placebo-controlled trial. J Invest Dermatol. 2014 Dec;150(12):1331-5.[Abstract]
36. Beissert S, Werfel T, Frieling U, et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus. Arch Dermatol. 2006 Nov;142(11):1447-54.[Abstract]
37. Kawashita MY, Tsai K, Aoiki V, et al. Mycophenolate mofetil as an adjuvant therapy for classic and endemic pemphigus foliaceus. J Dermatol. 2005 Jul;32(7):574-80.[Abstract]
38. Joly P, Mouquet H, Roujeau JC, et al. A single cycle of rituximab for the treatment of severe pemphigus. N Engl J Med. 2007 Aug 9;357(6):545-52.[Abstract][Full Text]
39. Ahmed AR, Spigelman Z, Cavacini LA, et al. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med. 2006 Oct 26;355(17):1772-9.[Abstract][Full Text]
40. Gurcan HM, Jeph S, Ahmed AR, et al. Intravenous immunoglobulin therapy in autoimmune mucocutaneous blistering diseases: a review of the evidence for its efficacy and safety. Am J Clin Dermatol. 2010;11(5):315-26.[Abstract]
41. Behzad M, Möbs C, Kneisel A, et al. Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult-to-treat pemphigus vulgaris. Br J Dermatol. 2012 Apr;166(4):844-52.[Abstract]
42. Frew JW, Murrell DF. Current management strategies in paraneoplastic pemphigus (paraneoplastic autoimmune multiorgan syndrome). Dermatol Clin. 2011 Oct;29(4):607-12.[Abstract]
43. Czernik A, Camilleri M, Pittelkow MR, et al. Paraneoplastic autoimmune multiorgan syndrome: 20 years after. Int J Dermatol. 2011 Aug;50(8):905-14.[Abstract]
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45. Werth VP. Treatment of pemphigus vulgaris with brief, high-dose intravenous glucocorticoids. Arch Dermatol. 1996 Dec;132(12):1435-9.[Abstract]
46. US National Library of Medicine. Open-label Study to Determine the Maximum Tolerated Dose of DSG3-CAART in Mucosal-dominant PV Patients (mPV). Jan 2021 [internet publication].[Full Text]
47. Ren Z, Narla S, Hsu DY, et al. Association of serious infections with pemphigus and pemphigoid: analysis of the Nationwide Inpatient Sample. J Eur Acad Dermatol Venereol. 2018 Oct;32(10):1768-76.[Abstract]
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