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

Dedee Murrell , Branka Marinovic , Frédéric Caux (1) , Catherine Prost , Razzaque Ahmed , Katarzyna Wozniak , Masayuki Amagai , Johann Bauer , Stefan Beissert (2) , Luca Borradori (3) , Donna Culton , Janet Fairley , David Fivenson , Marcel Jonkman , M Peter Marinkovich , David Woodley , John Zone , Valeria Aoki , Philippe Bernard (4) , Leena Bruckner-Tuderman (5) , Giuseppe Cianchini , Vanessa Venning , Luis Diaz (6) , Rudiger Eming , Sergei Grando , Russell Hall , Takashi Hashimoto , Josep Herrero-González , Michael Hertl , Pascal Joly (7, 8) , Sarolta Karpati , Jaehwan Kim , Soo Chan Kim , Neil Korman , Cezary Kowalewski , Sang Eun Lee , David Rubenstein , Eli Sprecher (9) , Kim Yancey , Giovanna Zambruno (10) , Detlef Zillikens , Serge Doan (11) , Benjamin Daniel , Victoria Werth

1 Service de dermatologie [Avicenne]
2 Department of Dermatology
3 UNIBE - Universität Bern [Bern]
4 ICA - Immunodermatologie, Cytokines, Cancer - EA 7319
5 Dermatology
6 XLIM-OSA - OSA
7 Service de dermatologie [Rouen]
8 PANTHER - Physiopathologie, Autoimmunité, maladies Neuromusculaires et THErapies Régénératrices
9 Laboratory of Molecular Dermatology and Department of Dermatology
10 Molecular and Cell Biology Laboratory
11 Service d'ophtalmologie [Hopital Bichat - Paris]

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.

Keywords: mucous membrane pemphigoid; definitions; consensus; severity score; outcome measures

Domains: Life Sciences [q-bio]; Life Sciences [q-bio]; Human health and pathology; Life Sciences [q-bio]; Human health and pathology; Dermatology

Dedee Murrell, Branka Marinovic, Frédéric Caux, Catherine Prost, Razzaque Ahmed, et al.. Definitions and outcome measures for mucous membrane pemphigoid: Recommendations of an international panel of experts. Journal of The American Academy of Dermatology, 2015, 72 (1), pp.168-174. ⟨10.1016/j.jaad.2014.08.024⟩. ⟨hal-02377693⟩