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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.
Quick Reference
History & Exam
Key Factors
Other Factors
Diagnostics Tests
Treatment Options
Definition
Epidemiology
Etiology
Pathophysiology
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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]
Antiga E, Bech R, Maglie R, et al. S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2023 Jun;37(6):1118-34.[Abstract][Full Text]
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][Full Text]
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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32. Ouedraogo E, Gottlieb J, de Masson A, et al. Risk factors for death and survival in paraneoplastic pemphigus associated with hematologic malignancies in adults. J Am Acad Dermatol. 2019 Jun;80(6):1544-9.[Abstract]
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