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 characterised 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 desmogleins 1 and 3. The diagnosis is based on histopathological examination, immunofluorescence microscopy and enzyme-linked immunosorbent assays (ELISA). Treatment of pemphigus vulgaris comprises systemic corticosteroids, together with adjuvant immunosuppressive drugs and/or rituximab.
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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. (2016). Pemphigus Vulgaris. In: Jonkman, M. (eds) Autoimmune Bullous Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-23754-1_8
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DOI: https://doi.org/10.1007/978-3-319-23754-1_8
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