ABSTRACT
Pemphigus vulgaris (PV) is an intraepidermal autoimmune blistering disease. It is characterized by the presence of painful oral erosions and flaccid blisters in the skin with a predilection for the scalp, face, and trunk. Cutaneous erosions that result from the rupture of blisters show a tendency for peripheral extension often leading to extensive areas of skin denudation. The diagnosis of pemphigus can be confirmed by immunofluorescence tests. Circulating antibodies can be detected quantitatively by desmoglein 1 and 3 enzyme-linked immunosorbent assay; the value of this test often correlates with disease activity. PV is potentially a life-threatening disease and is also associated with significant morbidity. Mortality rates in PV have been brought down from 90% in the presteroid era to less than 10%, with judicious use of steroids and immunosuppressive agents forming the mainstay of treatment. Recently, it has been shown that rituximab, a chimeric anti-CD20 monoclonal antibody, is effective as a first-line treatment and may further improve the outcome of the disease in the future.
