Beyond the skin: disease parameters in pemphigus

Area of Science:

• Dermatology
• Autoimmunology
• Clinical Research

Background:

• Pemphigus is a rare group of autoimmune bullous diseases affecting skin and mucous membranes.
• The chronic and recurring nature of pemphigus leads to significant morbidity, mortality, and impaired quality of life (QOL).
• Painful blisters and erosions, alongside therapeutic interventions and psychological factors like anxiety and depression, negatively impact patient QOL.

Purpose of the Study:

• To highlight the need for objective scoring systems to evaluate pemphigus disease activity.
• To correlate clinical disease activity with the quality of life (QOL) in pemphigus patients.
• To discuss current assessment tools for pemphigus and their suitability for clinical trials.

Main Methods:

• Review of current global scales used for assessing pemphigus clinical activity, including Autoimmune Bullous Skin Disorder Intensity Score (ABSIS), Pemphigus Disease Area Index (PDAI), and Pemphigus Visual Activity Scale (PVAS).
• Discussion of the most frequently used score for evaluating generic patient QOL, the Dermatology Life Quality Index (DLQI).
• Identification of the preferred QOL assessment tool in sponsored pemphigus clinical trials, the ABQOL, as opposed to DLQI.

Main Results:

• Established scales like ABSIS, PDAI, and PVAS are utilized to gauge pemphigus disease severity.
• The Dermatology Life Quality Index (DLQI) is commonly used for general QOL assessment.
• Sponsored clinical trials in pemphigus predominantly employ the ABQOL for QOL evaluation over the DLQI.

Conclusions:

• Objective assessment of pemphigus activity and its impact on QOL is crucial due to the disease's chronic nature.
• The selection of appropriate QOL scoring systems, such as ABQOL in clinical trials, is important for consistent and relevant patient outcome evaluation.
• Further research may be needed to refine and validate scoring systems that accurately capture the multifaceted impact of pemphigus on patient well-being.