Pemphigus: Pathogenesis to Treatment

Abstract

Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. These lesions correlate histologically with splits forming in the epidermis, leaving a blister roof composed of a few cell layers. Standard treatment of pemphigus involves oral corticosteroids, often with the addition of adjuvant therapies, to improve disease control, minimize corticosteroids side-effects, and increase the odds of remission. [Full article available at http://rimed.org/rimedicaljournal-2016-12.asp].

Keywords: corticosteroids; desmoglein 1; desmoglein 3; paraneoplastic pemphigus; pemphigus foliaceus; pemphigus vulgaris.