Skin-related quality of life (SRQL) in autoimmune bullous disease (AIBD) may be different between diseases and flare states. We sought to evaluate SRQL during patient-reported flare and non-flare. We also examined Skindex-16 construct validity as development cohorts did not contain AIBD. Diagnoses included: pemphigus, pemphigoid, dermatitis and herpetiformis (DH). Skindex-16 assessed SRQL (three domains [emotions, symptoms, functioning] scored 0-100, higher scores = worse SRQL). Differences in median Skindex-16 score were calculated with quantile regression. Internal consistency and standard error of measurement (SEM) were calculated using Cronbach's alpha. Convergent validity between Skindex-16 and other patient reported outcomes was assessed with Spearman's rank correlation. We identified 148 AIBD patients not experiencing flare and 65 experiencing flare. SRQL was worse for all conditions during flare than non-flare (SRQL difference > SEM and p<0.05). Desmoglein-1 and 3 were likewise significantly different between flare and non-flare, whereas other labs were not associated with flare. Compared with pemphigus, non-flare emotional SRQL was worse in pemphigoid (+7; 95% CI -0.41-14.4), and DH (+14.3; 3.62-25.0). During flare, pemphigoid had better emotional- (-14; -45.1-16.6) and symptom-related (-25; -53.6-3.64) SRQL. Cronbach's alpha was >0.80 for all domains and disease states. Skindex-16 showed moderate correlation with PROMIS Depression and patient-reported disease severity. Low correlation was seen with PROMIS Physical Functioning. Overall floor effects >20% were seen during non-flare, but ceiling effects were rare (<10% for all domains). SRQL was severely impacted by AIBD, especially during flare. Patients with pemphigoid and DH had worse SRQL during non-flare, highlighting possible therapeutic gap. These data support the construct validity of Skindex-16 in patients with AIBD.
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