Refractory pemphigus vulgaris: Treatment options

Abstract

Pemphigus vulgaris is an autoimmune mucocutaneous blistering disorder characterized by the presence of pathogenic autoantibodies against adhesion proteins on epidermal keratinocytes called desmogleins (DSG1 and DSG 3). It has a chronic relapsing and remitting course and in severe cases can be life threatening. Those patients who fail to respond to traditional treatments are considered to have refractory pemphigus. This review focuses on various therapies that are currently in practice as well as in pre-clinical or clinical trials for steroid-resistant pemphigus vulgaris.