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Definitions and outcome measures for mucous membrane pemphigoid: Recommendations of an international panel of experts - 13/12/14

Doi : 10.1016/j.jaad.2014.08.024 
Dedee F. Murrell, MA, BMBCh, MD, FACD a, , Branka Marinovic, MD, PhD b, Frederic Caux, MD, PhD c, Catherine Prost, MD, PhD d, Razzaque Ahmed, MD, DSc e, Katarzyna Wozniak, MD f, Masayuki Amagai, MD, PhD g, Johann Bauer, MD h, Stefan Beissert, MD i, Luca Borradori, MD j, Donna Culton, MD k, Janet A. Fairley, MD l, m, David Fivenson, MD n, Marcel F. Jonkman, MD, PhD o, M. Peter Marinkovich, MD p, q, David Woodley, MD r, John Zone, MD s, Valeria Aoki, MD, PhD t, Philippe Bernard, MD, PhD u, Leena Bruckner-Tuderman, MD, PhD v, Giuseppe Cianchini, MD w, x, Vanessa Venning, FRCP y, Luis Diaz, MD k, Rudiger Eming, MD z, Sergei A. Grando, MD, PhD, DSc aa, ab, ac, Russell P. Hall, MD ad, Takashi Hashimoto, MD, PhD ae, Josep E. Herrero-González, MD af, Michael Hertl, MD ag, Pascal Joly, MD, PhD ah, ai, Sarolta Karpati, MD, PhD, DrSc aj, Jaehwan Kim, MD, PhD a, ak, Soo Chan Kim, MD, PhD al, Neil J. Korman, MD, PhD am, Cezary Kowalewski, MD f, Sang Eun Lee, MD, PhD an, David R. Rubenstein, MD, PhD k, Eli Sprecher, MD, PhD ao, Kim Yancey, MD ap, Giovanna Zambruno, MD x, Detlef Zillikens, MD, PhD aq, Serge Doan, MD ar, as, Benjamin S. Daniel, BA, BCom, MBBS a, Victoria P. Werth, MD at, au
a Department of Dermatology at St George Hospital, University of New South Wales, Sydney, Australia 
b Department of Dermatology and Venereology, Zagreb University Hospital Center and School of Medicine, Zagreb, Croatia 
c Department of Dermatology, Avicenne Hospital, University Paris 13, Bobigny, France 
d Department of Dermatology, Department of Histology, Reference Center for Autoimmune Bullous Diseases, Avicenne Hospital, University Paris 13, Bobigny, France 
e Center For Blistering Diseases, Boston, Massachusetts 
f Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland 
g Department of Dermatology, Keio University School of Medicine, Tokyo, Japan 
h Division of Molecular Dermatology, Department of Dermatology, Paracelsus Medical University Salzburg, Salzburg, Austria 
i Department of Dermatology, University of Dresden, Dresden, Germany 
j Department of Dermatology, University Hospital of Bern, Bern, Switzerland 
k Department of Dermatology, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 
l Department of Dermatology, University of Iowa, Iowa City, Iowa 
m Department of Veterans Affairs Medical Center, Iowa City, Iowa 
n St Joseph Mercy Health System, Department of Dermatology, Ann Arbor, Michigan 
o University Medical Center Groningen, University of Groningen, Groningen, The Netherlands 
p Department of Dermatology, Stanford University School of Medicine, Stanford, California 
q Center for Clinical Sciences Research, and Division of Dermatology, Department of Veterans Affairs Palo Alto Healthcare System, Palo Alto, California 
r Department of Dermatology, Keck School of Medicine, University of Southern California, Los Angeles, California 
s Department of Dermatology, School of Medicine, University of Utah, Salt Lake City, Utah 
t Department of Dermatology, University of Sao Paulo, Sao Paulo, Brazil 
u Department of Dermatology, Reims University Hospital, University of Champagne-Ardenne, Reims, France 
v Department of Dermatology, University Freiburg Medical Center, Freiburg, Germany 
w Department of Immunodermatology, Istituto Dermopatico dell'Immacolata, IRCCS, Rome, Italy 
x Laboratory of Molecular and Cell Biology, Istituto Dermopatico dell'Immacolata, IRCCS, Rome, Italy 
y Department of Dermatology, Churchill Hospital, Oxford, United Kingdom 
z Department of Dermatology and Allergology, University Hospital, Philipps-Universität Marburg, Marburg, Germany 
aa Department of Dermatology, University of California, Irvine, California 
ab Department of Biological Chemistry Cancer Center, University of California, Irvine, California 
ac Research Institute, Institute for Immunology, University of California, Irvine, California 
ad Division of Dermatology, Duke Medical Center, Durham, North Carolina 
ae Kurume University School of Medicine, Kurume, Japan 
af Department of Dermatology, Hospital del Mar, Institut Hospital del Mar d'Investigacions Mèdiques, Barcelona, Spain 
ag Department of Dermatology, University Hospital, Marburg, Germany 
ah Clinique Dermatologique, Institut National de la Sante et de la Recherche Medicale (INSERM), INSERM U905, Rouen University Hospital, University of Rouen, Rouen, France 
ai Dermatology Department, Rouen University Hospital, University of Rouen, Rouen, France 
aj Department of Dermatology, Venereology, and Dermatooncology of the Semmelweis University, Budapest, Hungary 
ak Laboratory for Investigative Dermatology, Rockefeller University, New York, New York 
al Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Gangnam Severance Hospital, Seoul, Korea 
am Department of Dermatology and the Murdough Family Center for Psoriasis, University Hospitals Case Medical Center, Cleveland, Ohio 
an Department of Dermatology, CHA Bundang Medical Center, CHA University, Seongnam, Korea 
ao Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel 
ap Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas 
aq Department of Dermatology, University of Lubeck, Lubeck, Germany 
ar Department of Ophthalmology, Hopital Bichat, Paris, France 
as Fondation A de Rothschild, Paris, France 
at Philadelphia Department of Veterans Affairs Medical Center, University of Pennsylvania, Philadelphia, Pennsylvania 
au Department of Dermatology, University of Pennsylvania, Philadelphia, Pennsylvania 

Reprint requests: Dedee F. Murrell, MA, BMBCh, MD, FACD, Department of Dermatology, St George Hospital, University of New South Wales, Gray St, Kogarah, Sydney, N8W 2317 Australia.

Abstract

Mucous membrane pemphigoid encompasses a group of autoimmune bullous diseases with a similar phenotype characterized by subepithelial blisters, erosions, and scarring of mucous membranes, skin, or both. Although knowledge about autoimmune bullous disease is increasing, there is often a lack of clear definitions of disease, outcome measures, and therapeutic end points. With clearer definitions and outcome measures, it is possible to directly compare the results and data from various studies using meta-analyses. This consensus statement provides accurate and reproducible definitions for disease extent, activity, outcome measures, end points, and therapeutic response for mucous membrane pemphigoid and proposes a disease extent score, the Mucous Membrane Pemphigoid Disease Area Index.

Le texte complet de cet article est disponible en PDF.

Key words : consensus, definitions, mucous membrane pemphigoid, outcome measures, severity score

Abbreviations used : BPDAI, MMP, MMPDAI, PDAI


Plan


 The International Pemphigus and Pemphigoid Foundation generously supported hiring rooms at the Annual Meetings of the American Academy of Dermatology and European Academy of Dermatology and Venereology as well as audiovisual equipment and open access. The Korean World Congress Committee and the Society for Investigative Dermatology provided meeting rooms. This material is based on work supported by the Department of Veterans Affairs (Veterans Health Administration, Office of Research and Development, Biomedical Laboratory Research and Development) and by the National Institutes of Health (NIH K24-AR 02207) to Dr Werth.
 Conflicts of interest: None declared.


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