Review
Definitions and outcome measures for bullous pemphigoid: Recommendations by an international panel of experts

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Our scientific knowledge of bullous pemphigoid (BP) has dramatically progressed in recent years. However, despite the availability of various therapeutic options for the treatment of inflammatory diseases, only a few multicenter controlled trials have helped to define effective therapies in BP. A major obstacle in sharing multicenter-based evidences for therapeutic efforts is the lack of generally accepted definitions for the clinical evaluation of patients with BP. Common terms and end points of BP are needed 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. These recommendations from the International Pemphigoid Committee represent 2 years of collaborative efforts to attain mutually acceptable common definitions for BP and proposes a disease extent score, the BP Disease Area Index. These items should assist in the development of consistent reporting of outcomes in future BP reports and studies.

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Purpose

The purpose of this statement is to provide appropriate definitions for the various stages of disease activity, define therapeutic end points in BP, and to propose an objective disease extent measure that can be used in clinical trials. The use of the same definitions and outcome measures makes the results of trials more comparable. Since definitions and outcome measures for pemphigus2, 3, 4 have been published, most trials in pemphigus and reports have begun adopting these systems or referring

Methods

An international BP definitions committee was organized in 2008, at the point when the international pemphigus definitions committee completed its similar work on pemphigus.2 The committee was an expansion of the first committee and convened 7 times over 2 years to discuss the appropriate definitions. These meetings were held at the American Academy of Dermatology (AAD) annual meeting in San Antonio, TX, in 2009 (D. F. M. and V. P. W.); European Society for Dermatologic Research in Budapest,

Observation points

The end points are illustrated and summarized (Fig 1 and Table I).

Early end points

“Baseline” is the point at which a physician starts treatment for BP.

“Control of disease activity” (disease control; beginning of consolidation phase) is defined as the point at which new lesions or pruritic symptoms cease to form and established lesions begin to heal. The time to disease control is the time between baseline and this control point.

“End of the consolidation phase” is defined as the time at which no new lesions or

Discussion and conclusion

Despite many trials evaluating therapeutic options for BP, it has been difficult to compare the results from these trials because of the large number of end points and definitions of disease. The formation of an international committee of bullous disease experts able to meet face to face on a regular basis has provided a mechanism for developing agreement on these issues for BP. This statement with agreed-upon common definitions, and the ongoing discussion and refinement of proposed common

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The International Pemphigus and Pemphigoid Foundation generously supported renting rooms at the American Academy of Dermatology and audiovisual equipment; the European Society for Dermatological Research and European Academy of Dermatology provided meeting rooms. This report was supported in part by a grant from the National Institutes of Health (K24-AR 02207) to Dr Werth.

Conflicts of interest: None declared.

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