Table III.
Summary, by instrument, of assessments, reliability, and internal consistency
| Instrument or sub-score (reference range) | Mean ± SD | Range | Interobserver reliability (ICC) | Intraobserver reliability (ICC) | Cronbach's alpha | Shapiro-Wilk test of normality∗ |
|---|---|---|---|---|---|---|
| EBDASI (0-506) | 66.6 ± 61.4 | 1-256 | 0.942 | 0.99 (0.98-1) | 0.995 | 0.880 |
| Total activity (0-276) | 17.2 ± 17.6 | 0-89 | 0.867 | 0.96 (0.88-0.99) | 0.974 | 0.841 |
| A1 Skin (0-120) | 11.3 ± 11.1 | 0-49 | 0.903 | 0.96 (0.87-0.99) | 0.984 | 0.875 |
| A2 Scalp (0-10) | 1.2 ± 2.0 | 0-9 | 0.924 | 0.98 (0.94-0.99) | 0.972 | 0.880 |
| A3 Mucosa (0-120) | 2.4 ± 6.7 | 0-45 | 0.767 | 0.93 (0.77-0.98) | 0.781 | 0.648 |
| A4 Nails (0-20) | 1.5 ± 3.4 | 0-15 | 0.173 | 0.99 (0.98-1) | 0.383 | 0.359 |
| A5 Other (0-6) | 0.8 ± 1.1 | 0-4 | 0.575 | 0.74 (0.13-0.92) | 0.895 | 0.504 |
| Total damage (0-230) | 49.4 ± 45.6 | 1-182 | 0.935 | 0.99 (0.99-1) | 0.994 | 0.649 |
| iscorEB-clinician (0-114) | 11.0 ± 13.4 | 0-71.5 | 0.852 | 0.99 (0.98-1) | 0.981 | 0.872 |
| A Skin (0-60) | 5.8 ± 7.7 | 0-42.5 | 0.829 | 0.99 (0.96-1) | 0.939 | 0.930 |
| B Mucosal (0-15) | 1.2 ± 1.4 | 0-6 | 0.617 | 0.93 (0.77-0.98) | 0.909 | 0.741 |
| C Internal organ (0-12) | 1.4 ± 2.9 | 0-11 | 0.795 | 0.99 (0.97-1) | 0.993 | 0.789 |
| D Laboratory abnormalities (0-15) | 2.0 ± 3.7 | 0-13 | 0.860 | 1 (1-1) | 0.982 | 0.566 |
| E Complications/procedures (0-18) | 0.5 ± 0.8 | 0-5 | 0.473 | 0.87 (0.55-0.96) | 0.725 | 0.593 |
| QOLEB (0-51) | 16.6 ± 8.7 | 4-35 | 0.828 | |||
| iscorEB-patient (0-120) | 25.1 ± 13.6 | 2-48 | 0.931 |
EBDASI, Epidermolysis Bullosa Disease Activity and Scarring Index; ICC, intraclass correlation coefficient; iscorEB, Instrument for scoring the clinical outcomes of research for Epidermolysis Bullosa; QOLEB, Quality of Life in Epidermolysis Bullosa; SD, standard deviation.
∗
P-values for Shapiro-Wilk test are not provided as no variables were normally distributed.