Bullous pemphigoid and pemphigus vulgaris

Autoimmune bullous diseases are characterised by blistering of the skin or mucous membranes.1 Blisters form due to the antibodies against structural skin components. The two most common bullous diseases are bullous pemphigoid and pemphigus vulgaris. Most of our epidemiological data comes from European studies. Estimates of the incidence of bullous pemphigoid range from 2.8 per 100 000 person years in the US2 to 4.28 per 100 000 person years in the UK3 most commonly presenting in people over 80 years old. Pemphigus vulgaris is rarer and its geographic distribution more variable: whereas in Israel the incidence has been estimated at 5.3 per 100 000 person years, in the UK it was only 0.7.4

To the generalist these may seem to be niche diseases and are often overlooked or misdiagnosed, leading to poor patient outcomes. Because of their chronic nature, high morbidity and mortality, and the systemic effects from treatment, affected people require intensive management. In the past few decades great strides have been made in understanding these diseases but and the development of novel treatments, however these treatments themselves are associated with substantial disease burden. This article highlights the pathophysiology, investigations, and management of bullous pemphigoid and pemphigus vulgaris.