Pemphigus Vulgaris Scoring Systems: A Scoping Review

Area of Science:

• Dermatology
• Autoimmune Diseases
• Clinical Research Methodology

Background:

• Pemphigus vulgaris (PV) is a rare autoimmune blistering disease causing mucocutaneous lesions.
• Accurate assessment of PV disease activity, severity, and treatment response is vital for patient management and research.
• Existing clinical scoring systems for PV lack universal consensus on optimal use.

Purpose of the Study:

• To identify, evaluate, and summarize clinical scoring systems for pemphigus vulgaris.
• To assess the validity, reliability, and clinical applicability of these scoring systems.
• To inform the development of improved assessment tools for PV.

Main Methods:

• A comprehensive literature search was performed across major electronic databases and grey literature.
• Studies describing clinical scoring systems for PV were identified.
• Data extracted included scoring system components, validity, clinical applicability, and limitations.

Main Results:

• Several PV scoring systems were identified, including the Pemphigus Disease Area Index and Autoimmune Bullous Skin Disorder Intensity Score.
• Significant variations exist among systems regarding assessment domains (mucosal vs. cutaneous involvement, patient-reported outcomes, usability).

Conclusions:

• Current PV scoring systems offer frameworks but show variability in scope, validation, and implementation.
• Future systems should integrate biomarkers and quality of life measures, covering all clinical sites.
• A consensus on a universal scoring system is needed to address the complex nature of PV.