Definitions and outcome measures for mucous membrane pemphigoid: recommendations of an international panel of experts

Murrell, Dedee F. and Marinović, Branka and Caux, Frederic and Prost, Catherine and Ahmed, Razzaque and Wozniak, Katarzyna and Amagai, Masayuki and Bauer, Johann and Beissert, Stefan and Borradori, Luca and Culton, Donna and Fairley, Janet A. and Fivenson, David and Jonkman, Marcel F. and Marinkovich, M. Peter and Woodley, David and Zone, John and Aoki, Valeria and Bernard, Philippe and Bruckner-Tuderman, Leena and Cianchini, Giuseppe and Venning, Vanessa and Diaz, Luis and Eming, Rudiger and Grando, Sergei A. and Hall, Russell P. and Hashimoto, Takashi and Herrero-González, Josep E. and Hertl, Michael and Joly, Pascal and Karpati, Sarolta and Kim, Jaehwan and Chan Kim, Soo and Korman, Neil J. and Kowalewski, Cezary and Lee, Sang Eun and Rubenstein, David R. and Sprecher, Eli and Yancey, Kim and Zambruno, Giovanna and Zillikens, Detlef and Doan, Serge and Daniel, Benjamin S. and Werth, Victoria P. (2015) Definitions and outcome measures for mucous membrane pemphigoid: recommendations of an international panel of experts. Journal of the American Academy of Dermatology, 72 (1). pp. 168-174. ISSN 0190-9622

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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.

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