A major obstacle in performing multicenter controlled trials for pemphigus is the lack of a validated disease activity scoring system. Here we assess the reliability and convergent validity of the PDAI (pemphigus disease area index). A group of 10 dermatologists scored 15 patients with pemphigus to estimate the inter- and intra-rater reliability of the PDAI and the recently described ABSIS (autoimmune bullous skin disorder intensity score) instrument. To assess convergent validity, these tools were also correlated with the Physician’s Global Assessment (PGA). Reliability studies demonstrated an intra-class correlation coefficient (ICC) for inter-rater reliability of 0.76 [95% CI = 0.61–0.91] for the PDAI and 0.77 [0.63–0.91] for the ABSIS. The tools differed most in reliability of assessing skin activity, with an ICC of 0.39 [0.17–0.60] for the ABSIS and 0.86 [0.76–0.95] for the PDAI. Intra-rater test-retest reliability demonstrated an ICC of 0.98 [0.96–1.0] for the PDAI and 0.80 [0.65–0.96] for the ABSIS. The PDAI also correlated more closely with the PGA. We conclude that the PDAI is more reproducible and correlates better with physician impression of extent. Subset analysis suggests that for this population of mild to moderate disease activity, the PDAI captures more variability in cutaneous disease than the ABSIS.

Background-Little is known about quality of life in patients with cutaneous lupus erythematosus.

Objective: To determine how to use the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) to classify patients according to disease severity (mild, moderate, and severe) and to identify which patients respond to therapy.Design: Cohort.

Background-Reliable and validated measures of skin disease severity are needed for cutaneous dermatomyositis (DM). CDASI (Cutaneous Dermatomyositis Disease Area and Severity Index), DSSI (Dermatomyositis Skin Severity Index), and CAT (Cutaneous Assessment Tool) skin indices have been developed as outcome instruments.

Background-Quality of life (QoL) for patients with inflammatory skin disease can be significant, but has been evaluated in just one study in dermatomyositis (DM).