Table I.
Demographic and clinical characteristics of the study cohort∗
Age | |
Range (mean ± SD) | 16-73 (43.7 ± 14.6) |
Sex | |
Female | 4 (27) |
Race/ethnicity | |
Caucasian | 11 (73) |
Asian | 1 (7) |
Middle-eastern | 3 (20) |
Epidermolysis Bullosa type, subtype | |
Simplex, localized | 4 (27) |
Junctional, generalized intermediate | 2 (13) |
Dominant dystrophic | 6 (40) |
Recessive dystrophic, generalized intermediate | 2 (13) |
Recessive dystrophic, generalized severe | 1 (7) |
Disease severity; corresponding EBDASI score range12 | |
Mild; 0-42 | 6 (40) |
Moderate; 43-106 | 6 (40) |
Severe; 107-506 | 3 (20) |
Disease severity; iscorEB validation criteria5 | |
Mild; localized involvement, 0 non-skin complications | 6 (40) |
Moderate; widespread involvement, <3 non-skin complications | 4 (27) |
Severe; generalized involvement, ≥3 non-skin complications | 5 (33) |
EBDASI, Epidermolysis Bullosa Disease Activity and Scarring Index; iscorEB, Instrument for scoring the clinical outcomes of research for Epidermolysis Bullosa; SD, standard deviation.
All values are n (%) unless otherwise specified.