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Management of Pemphigus and Pemphigoid

Joint Meeting of the EADV Task Force on Autoimmune Bullous Diseases and the International Pemphigus & Pemphigoid Foundation


During the last EADV congress held in Paris (September 17th to September 20th, 2008) the EADV task force for autoimmune blistering diseases presided by IPPF Medical Advisory Board Member Prof. Marcel F. Jonkman (Groningen, NL) and Prof. Luca Borradori (Berne, CH) organized the first satellite meeting of experts, practising clinicians and young dermatologists-in-training interested in this wide and challenging area.

The International Pemphigus & Pemphigoid Foundation, the pre-eminent global organization dedicated to improving the quality of life of all people diagnosed with, or affected by, pemphigus or pemphigoid, joined the EADV task force, with the help of IPPF MAB Chairman Prof. Jean-Claude Bystryn (New York, USA). The final goal was to address and review clinical and management issues and also practical problems in the daily life of affected patients.

In the first part of the meeting, Prof. Borradori discussed how to make the diagnosis of pemphigus and pemphigoid (and the common pitfalls) with a review of the immunopathological criteria required for diagnosis. In this context, he emphasized the difficulty of classifying some elderly patients with chronic pruritic skin eruptions because of the lack of well accepted criteria for bullous pemphigoid, a problem that needs to be urgently addressed. [Editor: See related story, page 1]

Prof. J.C Roujeau (Paris, FR) subsequently presented data about epidemiology and incidence of these diseases with an analysis of the conflicting results existing about the morbidity and mortality rates of bullous pemphigoid between European and USA centers. IPPF MAB Member Prof. Dedee Murrell (Sydney, Australia) discussed how essential it is to use common terms and end points so that experts in the field can accurately measure and assess disease extent, activity, severity, and therapeutic response, and thus facilitate and advance clinical trials. The IPPF recently funded and completed such a consensus paper on such definitions in pemphigus but for pemphigoid there is still a lot to do.

The second part of the meeting was focused on the general care of autoimmune bullous diseases in the USA (Prof. Bystryn) and in Europe (Prof. Jonkman). There are major differences in the management of affected patients that are determined not only by the profoundly different health care systems, but also by the lack of good studies and evidence-based guidelines, particularly in pemphigus.

{quotes align=right}In a third round of presentations, IPPF MAB Members Prof. Michael Hertl (Marburg, GE) and Prof. Pascal Joly (Rouen, FR) described their encouraging experiences with the use of rituximab, an anti-CD20 monoclonal antibody, in the treatment of severe pemphigus patients.{/quotes} The question arose whether such biological treatment should be used in patients as first-line therapy with the aim of inducing a definite remission in a significant percentage of patients without the side effects from prolonged therapy with systemic corticosteroids and classic immunosuppressive agents. Controlled studies are needed to justify this choice.

Prof. Zillikens (L.beck, D) reviewed the therapeutic ladder followed to manage patients affected by bullous pemphigoid in Germany, emphasizing the difficulty in certain patients where the use of intravenous immunoglobulin, rituximab and or immunoadsorption are required to control the disease. Dr. D. Mimouni (Israel) reviewed the properties of an immunosuppressive agent, mycophenolate mofetil, which has gained a place in the management of blistering diseases in recent years. Although this drug has certain advantages (more selective effect on lymphocytes and better side effect profile) it is unclear if this molecule is really superior to azathioprine, a much cheaper and well established immunosuppressant.

The final and very important part of the meeting was dedicated to the patients perspectives of these diseases. The IPPF’s European Spokesman Mr. Peter Foldes (France) shared his medical experience with pemphigus (he had been diagnosed with this disease many years ago). He described his impressions and the insecurity he experienced concerning therapy side effects, disease evolution and prognosis. He emphasized the need to share knowledge and experience about the disease with other affected patients and the importance of having patients organizations such as the IPPF available that can support and properly inform people living with pemphigus and other autoimmune blistering disorders.

In the whole, the satellite symposium was a definite success. The number of participants (more than 120) far exceeded expectations. Every presentation was followed by an animated discussion  attesting to the friendly and stimulating atmosphere in the room  involving not only experts, but younger colleagues and dermatologists-in-training not yet familiar with this group of diseases. The take-home message for the organizers was clear: immediately start to think about the next task force meeting in the area of autoimmune bullous diseases with an attractive, practical and relevant program!


Posted in Issue 54 - Fall 2008


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