Abstract
Pemphigus is a group of chronic mucocutaneous blistering diseases caused by autoantibodies directed against the desmosomal cadherins desmoglein 1 and/or desmoglein 3 (Table 8.1). Pemphigus can be divided into two major forms, pemphigus foliaceus (PF) and pemphigus vulgaris (PV). The mucosal dominant form of PV is characterized by blistering of the mucous membranes and antibodies directed against desmoglein 3. Patients with mucocutaneous PV have blistering of both the mucous membranes and the skin, and the autoantibodies are directed against desmoglein 1 and 3. The diagnosis is based on histopathological examination, immunofluorescence microscopy and enzyme-linked immunosorbent assays (ELISA). First line treatment of pemphigus vulgaris is rituximab in combination with systemic corticosteroids with the treatment goal of achieving complete remission on/off therapy by 6 months.
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JAMA Dermatology Patient Page, Pemphigus http://archderm.jamanetwork.com/article.aspx?articleid=1879985
International Pemphigus & Pemphigoid Foundation http://www.pemphigus.org/
Van der Wier G. Acantholysis in pemphigus [dissertation]. Groningen: University of Groningen; 2014. http://irs.ub.rug.nl/ppn/38300196X
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van der Wier, G., Jonkman, M.F., Horváth, B. (2022). Pemphigus Vulgaris. In: Horváth, B. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-030-91557-5_8
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