Practice Clinical updates

Bullous pemphigoid and pemphigus vulgaris

BMJ 2017; 357 doi: https://doi.org/10.1136/bmj.j2169 (Published 08 June 2017) Cite this as: BMJ 2017;357:j2169
  1. Mahaz Kayani, education fellow1,
  2. Arif M Aslam, consultant dermatologist2
  1. 1Lancashire Teaching Hospitals NHS Foundation Trust, Royal Preston Hospital, Fulwood, Preston PR2 9HT, UK
  2. 2St Helens and Knowsley Teaching Hospitals NHS Trust, St Helens WA9 3DA, UK
  1. Corresponding author: A Aslam a.aslam{at}doctors.org.uk

What you need to know

  • Bullous pemphigoid and pemphigus vulgaris are diseases of an ageing population

  • Both are associated with high morbidity and mortality, with the commonest cause of death being opportunistic infections from prolonged immunosuppression

  • Topical and systemic corticosteroids are first line treatment for most patients

  • Steroid-sparing regimens are used where steroid treatment fails or if there are adverse effects

  • Non-specialists have important roles in both early detection and ongoing management of these diseases because of their chronic nature and the systemic effects of treatment

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.

What causes bullous pemphigoid and pemphigus vulgaris?

The aetiology of bullous pemphigoid and pemphigus vulgaris is poorly understood. Possible triggers for bullous pemphigoid include trauma as …

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