Pemphigus: Pathogenesis to Treatment

摘要

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 pem- phigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinical- ly these diseases present most often with epidermal ero- sions 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 con- trol, minimize corticosteroids side-effects, and increase the odds of remission.