What is pemphigus vulgaris?

Updated: Jun 14, 2018
  • Author: Bassam Zeina, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Pemphigus is derived from the Greek word pemphix meaning bubble or blister. Pemphigus describes a group of chronic bullous diseases, originally named by Wichman in 1791. The term pemphigus once included most bullous eruptions of the skin, but diagnostic tests have improved, and bullous diseases have been reclassified.

The term pemphigus refers to a group of autoimmune blistering diseases of the skin and mucous membranes characterized histologically by intraepidermal blister and immunopathologically by the finding of in vivo bound and circulating immunoglobulin G (IgG) antibody directed against the cell surface of keratinocytes. The 3 primary subsets of pemphigus include pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. [10] Each type of pemphigus has distinct clinical and immunopathologic features. Pemphigus vulgaris accounts for approximately 70% of pemphigus cases.


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