Pemphigus Vulgaris Scoring Systems: A Scoping Review.

Abstract

Background: Pemphigus vulgaris is a rare autoimmune blistering condition characterised by mucocutaneous lesions secondary to acantholysis. Assessment of disease activity, severity, and treatment response is crucial for guiding management and research. Multiple clinical scoring systems have been developed for pemphigus vulgaris; however, consensus on their optimal use is lacking. This scoping review aims to identify, evaluate, and summarise the clinical scoring systems used in pemphigus vulgaris, focusing on validity, reliability, and application in clinical practice and research.

Methods: A comprehensive literature search was conducted using electronic databases (PubMed (Medline), Embase, Web of Science, Scopus, and Cochrane Library) as well as grey literature to identify studies describing clinical scoring systems for pemphigus vulgaris. Data on scoring system components, validity, clinical applicability, and limitations were extracted and synthesised.

Results: The review identified several scoring systems, including the Pemphigus Disease Area Index, Autoimmune Bullous Skin Disorder Intensity Score, and other less commonly used tools. Scoring systems varied in design, with key differences noted in assessment domains, including mucosal versus cutaneous involvement, patient-reported outcomes, and usability.

Conclusion: Current clinical scoring systems for pemphigus vulgaris provide frameworks for disease assessment but exhibit variability in scope, validation, and practical implementation. Further development to incorporate emerging biomarkers and quality of life, as well as encompass all clinical subsites, will enhance their utility in guiding patient care and advancing research. This review highlights the need for consensus on a universal scoring system tailored to the multifaceted nature of pemphigus vulgaris.