Abstract
The disease pemphigus encompasses a group of related blistering conditions characterized by circulating antibodies against keratinocyte cell surface antigens important in mediating cell-to-cell adhesion (Becker and Gaspari, Dermatol Clin, 1993;11:429; Lever. J Am Acad Dermatol. 1979;1:2). Of the various types and forms of the disease including pemphigus foliaceus, pemphigus erythematosus, IgA pemphigus, and pemphigus vegetans, it is pemphigus vulgaris (PV) and paraneoplastic pemphigus (PP) that constitute the most important causes of mortality. Overall, these disorders are quite rare, with an estimated prevalence of between 1 in 100,000 for PV and less than 1 in 1,000,000 for PP. Both disorders are seen principally in aged adults with a near equal gender distribution. PV is more commonly observed among Jews and individuals of Mediterranean descent, whereas there is no known ethnic predilection for PP.
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References
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Kushner, J., Morgan, M.B. (2016). Pemphigus Vulgaris and Paraneoplastic Pemphigus. In: Crowe, D., Morgan, M., Somach, S., Trapp, K. (eds) Deadly Dermatologic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-31566-9_38
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DOI: https://doi.org/10.1007/978-3-319-31566-9_38
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