Autoimmune Blistering Diseases Part II—Diagnosis and Management









Dédée F. Murrell, MA, BMBCh, FAAD, MD, FACD, Guest Editor
When invited to edit a special issue of Dermatologic Clinics on Autoimmune Blistering Diseases by Bruce Thiers, I was delighted and honored to accept. I had just finished editing two issues on the genetic blistering disease, Epidermolysis Bullosa, for Dermatologic Clinics and found that the scientific and dermatologic community were keen to contribute to these theme-based issues. There have been a few excellent textbooks devoted to autoimmune blistering diseases (AIBD), with some focusing on the pathology and others on management, but the field is advancing rapidly. There was so much to cover about AIBD that it was decided to cover clinical features, diagnostic testing, and pathogenesis in the first issue and follow it with this issue on management.


Unlike a textbook, where the articles may be over a year or two out of date by the time the book is published, these articles have been written in the last 6 months and by respected leaders in the particular aspects of AIBD. The issue has been organized such that the drugs that are commonly used to treat AIBD are discussed in detail first by dermatologists familiar in their use for AIBD, including Stefan Beissert on azathioprine, Daniel Mimouni on mycophenolate mofetil, Vicky Werth on dapsone, and Pascal Joly on rituximab. This is followed by specific sections on the complications that can result from these treatments and how to manage them, overall by Amit Pandya and specifically on preventing infections by Julia Lehman, Michael Camilleri, and Amer Kalaaji. There is a special article devoted to issues of management of AIBD in pregnancy by Tess McPherson and Vanessa Venning. This is important because the major morbidity and mortality these patients have (apart from risk of blindness in mucous membrane pemphigoid) stem from the treatments themselves. It is not merely a matter of writing a prescription for oral steroids without considering what can be done from baseline to reduce the risks of significant side effects from corticosteroids.


Subsequently, a leading AIBD clinician or team has written about the practical aspects of treating each AIBD, including pemphigus vulgaris and foliaceus, paraneoplastic pemphigus, bullous pemphigoid, mucous membrane pemphigoid, pemphigoid gestationes, linear IgA disease, epidermolysis bullosa acquisita, bullous lupus, and dermatitis herpetiformis. These leaders include Luca Borradori, Russell Hall, Pascal Joly, Sarolta Karpati, Vanessa Venning, and Kim Yancey.


As AIBD are relatively rare skin diseases, it has been difficult to perform randomized controlled trials with sufficient power to demonstrate a statistically significant difference between treatments. To address this, an international group of AIBD experts has developed consensus definitions of the stages in the management of the AIBD and severity scoring systems which have been validated, so that even small clinical trials as well as larger ones can be combined using meta-analysis to determine significance. If we all use the same language and outcome measures in our case reports and case series on AIBD, we will be better able to compare the severity of AIBD patients and their responses to treatment. There are articles detailing the outcome measures of disease extent that have been validated so far as well as studies on quality of life.


Last, we have a section about the services and management of AIBD in various countries and the patient support group, the International Pemphigus and Pemphigoid Foundation. The countries where I could recruit experts willing and able to write about this include Australia, Austria (Martin Laimer, Gabi Pohla-Gubo, Johann Bauer, Helmut Hintner, and colleagues), Croatia (Branka Marinovich and Jasna Lipozencic), France (Nicolas Meyer, Carle Paul, and Pascal Joly), Germany (Enno Schmidt and Detlef Zillikens), Iran (Cheyda Chams Davatchi), Japan (Masa Amagai), and Spain (Ricardo Suárez, Agustín España, and José Mascaró Jr).


Unlike a textbook, these articles can be found on Medline and PubMed and are accessible online. The individual issues of the journal may be purchased for much less cost than either buying a textbook or the articles individually.


The contributors deserve many thanks for their time and effort in writing these articles at relatively short notice and in a succinct manner with excellent color photographs and figures. Inevitably, there are topics and experts whose contributions I would have liked to include but it is difficult for busy clinicians to find extra time to write these articles. On that note, I would particularly like to acknowledge and thank my current and former fellows, Benjamin Daniel, Lizbeth Intong, Linda Martin, and Supriya Venugopal, as well as my former research medical students, Shien-Ning Chee, Andrew Dermawan, John Frew, and Deshan Sebaratnam, who have all been doing projects on AIBD and who have written many of these articles with me. It is good for patients with AIBD if more young dermatologists take an interest in their condition.


Hopefully these two issues will be educational not only for dermatologists but also for all clinicians who interact with patients with AIBD, as well as scientists, family members, and the patients themselves. Understanding what is known so far about a disease leads to improved clinical practice, better research, and improved compliance with therapy.


Only gold members can continue reading. Log In or Register to continue

Stay updated, free articles. Join our Telegram channel

Feb 12, 2018 | Posted by in Dermatology | Comments Off on Autoimmune Blistering Diseases Part II—Diagnosis and Management

Full access? Get Clinical Tree

Get Clinical Tree app for offline access