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Pemphigus Disease Activity Measurements: Pemphigus Disease Area Index, Autoimmune Bullous Skin Disorder Intensity Score, and Pemphigus Vulgaris Activity Score

  • Ziba RahbarEmail author
Living reference work entry

Abstract

Pemphigus is a group of rare skin condition with blisters in the skin and mucosa that are associated with autoantibodies against intercellular adhesion molecules. Effective therapies can improve its devastating morbidities and mortality. Using a uniform, consensus-based and validated disease activity measure is essential to multicenter clinical trials for new therapies, meta-analysis of smaller studies, and evidence-based practice. However, above 116 outcome measures exist to assess pemphigus disease activity.

Pemphigus Disease Area Index (PDAI), Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), and Pemphigus Vulgaris Activity Score (PVAS) are the most comprehensively validated measures. The consensus-based standards for the selection of health status measurement instruments (COSMIN) study recommends investigation for the measurement properties of reliability, validity, and responsiveness. Here, PDAI, ABSIS, and PVAS are presented and compared based on their measurement properties including reliability, feasibility, and correlation with anti-desmoglein using one large prospective study (n = 100) comparing all three measures. Also, a review of additional properties including validity, responsiveness, and severity cutoffs is included using updated studies.

PDAI has the greatest reliability and is least affected by variability in rater’s pemphigus expertise. All three pemphigus measures have acceptable convergent validity with Physician Global Assessment, but PVAS seems to have the greatest value. PVAS is the fastest and simplest score to complete. Responsiveness studies may be needed for comprehensive validation. There are inconsistencies in severity cutoffs. Recently, PDAI, as the internationally accepted pemphigus disease measure, has been increasingly used in different studies around the world.

Keywords

Pemphigus Autoimmune Blistering Bullous Disease activity Disease damage Measure Score Severity PDAI 

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Autoimmune Bullous Diseases Research Center, Department of DermatologyRazi Hospital, Tehran University of Medical SciencesTehranIran
  2. 2.Dermatology DepartmentStanford University School of MedicinePalo AltoUSA

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