British Association of Dermatologists' Guidelines for the Management of Pemphigus Vulgaris 2017

K.E. Harman; D. Brown; L.S. Exton; R.W. Groves; P.J. Hampton; M.F. Mohd Mustapa; J.F. Setterfield; P.D. Yesudian


The British Journal of Dermatology. 2017;177(5):1170-1201. 

In This Article

Future Directions

As these guidelines illustrate, there is a lack of high-quality evidence supporting the use of many drugs in PV. Even answering the basic question of whether there is benefit in adding adjuvant immunosuppressants to corticosteroids is not clear-cut for most drugs. The answers to these questions will only come from large, multicentre RCTs, which would need to be of sufficient length to demonstrate the long-term outcomes that are of relevance in this chronic disease.

The role of biologics and their place in the management of PV is an area of great interest. Most experience comes from treating patients with established disease resistant to standard treatment. It is interesting to speculate whether using rituximab, or newer anti-CD20 drugs, as a first-line drug in newly presenting, treatment-naive patients might offer better long-term outcomes than the standard approach of corticosteroids with an adjuvant immunosuppressant. Such potential advantages might offset its additional cost in the long-term. A recent unblinded RCT has shown that rituximab combined with prednisolone is more effective than prednisolone only in newly diagnosed patients.[45] Further studies to confirm this result and to compare with corticosteroid–immunosuppressant combinations are awaited.

The development of anti-CD20 drugs that can be self-administered by subcutaneous injection also has the potential to be a very useful step forward. At present, ongoing trials using rituximab and of atumumab may help answer some of these questions, and positive results may lead to formal licensing, making use of these drugs more straightforward. In 2016, NHS England approved routine commissioning of rituximab in the treatment of pemphigus that has failed to respond to systemic steroids together with adjuvant immunosuppressive agents such as mycophenolate or azathioprine,[289] thereby ensuring consistent access and funding across the NHS in England. This document was produced prior to the recent RCT using rituximab.[45]

Further investment in diagnostic laboratories is needed to enable routine use of tests such as immunoprecipitation to enable more precise diagnosis of pemphigus subtypes leading to better targeted investigation and treatment.