Pemphigus: Complete Literature on Symptoms, Pathogenesis & Treatment

Dr. Jayesh Shah; Dr. Devendra Parmar

Volume : V, Issue : X, October - 2015

Pemphigus: Complete Literature on Symptoms, Pathogenesis & Treatment

Dr. Jayesh Shah, Dr. Devendra Parmar

Abstract :

Pemphigus is a group of rare autoimmune diseases. Such diseases occur when the body’s immune system attacks healthy cells. Mucous memanes are found in the mouth, nose, throat, eyes, and genitals. Pemphigus is a group of potentially life–threatening diseases characterized by cutaneous and mucosal blistering. There is a fairly strong genetic background to pemphigus with linkage to HLA class II alleles. Certain ethnic groups, such as Ashkenazi Jews and those of Mediterranean origin, are especially liable to pemphigus. Pemphigus vulgaris (PV), the most common and important variant, is an autoimmune blistering disease characterized by circulating pathogenic IgG antibodies against desmoglein 3 (Dsg3), about half the patients also having Dsg1 autoantibodies. Oral lesions are initially vesiculobullous but readily rupture, new bullae developing as the older ones rupture and ulcerate. Systemic corticosteroids remain the mainstay of therapy for pemphigus. Their use has transformed what was almost invariably a fatal illness into one whose mortality is now below 10%. Unfortunately, the high doses and prolonged administration of corticosteroids that are often needed to control the disease result in numerous side effects, many of which are serious or even life–threatening