Scoring systems for Mucous Membrane Pemphigoid – A review of the literature
Authors/Creators
- 1. University of New South Wales, Sydney, Australia
- 2. University of New South Wales, Sydney, Australia|St. George Clinical School, Sydney, Australia
Description
Mucous Membrane Pemphigoid (MMP) is a group of rare chronic autoimmune blistering diseases that predominantly affects one or more of the mucous membranes. Without treatment it can cause significant complications such as esophageal strictures, breathing difficulties, speech difficulties, laryngeal stenosis and blindness. Despite recent advances in targeted immunotherapy there have been no randomised controlled trials to date for MMP. Whilst this is partially a reflection of the rarity of the disease, it is also due to the lack of validated scoring systems. Validated scoring systems enable an investigator to effectively document response to treatment and communicate disease severity to peers. This article reviews and evaluates existing global and mucous membrane specific scoring systems for MMP. This article also proposes the creation of an Investigator's Global Assessment for MMP as a future area of research.
Why was the study undertaken? This scoping literature review was undertaken to provide an overview of the existing scoring systems for MMP. It critically analyses the strengths and weaknesses of the scoring systems and their validation studies, and points out any pathological associations with score results.
What does this study add? This study introduces the concept of an IGA score for MMP, and discusses its utility and value.
What are the implications of this study for the understanding of skin physiology and pathology and/or disease management? We hope this manuscript will aid in the creation of a validated IGA score, thus facilitating randomised controlled trials on MMP and FDA approval for MMP treatments.
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Additional details
References
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