Table 1.
Variable | Level | N | % | Mean FDLQI score | Median FDLQI score | P value* |
---|---|---|---|---|---|---|
Overall | 78 | 100 | 10.3 | 10.0 | ||
Sex | Male | 35 | 44.9 | 11.0 | 10.0 | |
Female | 43 | 55.1 | 9.8 | 10.0 | 0.698 | |
Age (years) | < 18 | 48 | 61.5 | 10.0 | 10.0 | |
≥ 18 | 30 | 38.5 | 10.8 | 10.5 | 0.837 | |
Clinical type | LIa | 68 | 87.2 | 10.2 | 10.0 | |
CIEb | 10 | 12.8 | 8.9 | 9.5 | 0.482 | |
Mutated gene | Undetermined | 33 | 42.3 | 9.5 | 9.0 | |
ABCA12 | 6 | 7.7 | 13.0 | 12.0 | ||
ALOX12B | 10 | 12.8 | 11.7 | 12.0 | ||
ALOXE3 | 2 | 2.6 | 6.5 | 6.5 | ||
CYP4F22 | 5 | 6.4 | 7.6 | 8.0 | ||
NIPAL4 | 4 | 5.1 | 6.2 | 7.5 | ||
SDR9C7 | 0 | 0 | NA | NA | ||
TGM1 | 18 | 23.1 | 10.3 | 10.0 | 0.163 | |
Clinical severity scored | Mild-moderate | 56 | 71.8 | 9.2 | 8.0 | |
Severe | 22 | 28.2 | 13.1 | 13.0 | 0.003 | |
Ear deformity | NO | 62 | 79.5 | 9.7 | 8.5 | |
YES | 16 | 20.5 | 12.8 | 13.0 | 0.016 | |
Ectropion | NO | 53 | 67.9 | 9.9 | 9.0 | |
YES | 25 | 32.1 | 11.3 | 12.0 | 0.128 | |
Eclabium | NO | 73 | 93.6 | 10.1 | 9.0 | |
YES | 5 | 6.4 | 14.0 | 13.0 | 0.069 | |
Thick scales | NO | 41 | 52.6 | 9.9 | 9.0 | |
YES | 37 | 47.4 | 10.9 | 12.0 | 0.336 | |
Dark scales | NO | 40 | 51.3 | 9.1 | 9.5 | |
YES | 38 | 48.7 | 11.6 | 12.0 | 0.094 | |
Face scales | NO | 22 | 28.2 | 8.5 | 7.5 | |
YES | 56 | 71.8 | 11.0 | 11.0 | 0.039 | |
Fissures | NO | 53 | 67.9 | 9.8 | 9.0 | |
YES | 25 | 32.1 | 11.4 | 11.0 | 0.209 | |
PPKc | NO | 21 | 26.9 | 8.6 | 6.0 | |
YES | 57 | 73.1 | 11.0 | 11.0 | 0.041 | |
Itch | NO | 9 | 11.5 | 9.6 | 11.0 | |
YES | 69 | 88.5 | 10.4 | 10.0 | 0.796 | |
Recurrent infections | NO | 60 | 76.9 | 9.5 | 8.0 | |
YES | 18 | 23.1 | 13.0 | 13.0 | 0.004 | |
Foul-smell | NO | 50 | 64.1 | 9.2 | 8.0 | |
YES | 28 | 35.9 | 12.4 | 12.0 | 0.009 | |
Walking problems | NO | 66 | 84.6 | 10.0 | 9.0 | |
YES | 12 | 15.4 | 12.4 | 12.5 | 0.048 | |
Visit/year | 1 | 33 | 42.3 | 10.5 | 9.0 | |
2 | 27 | 34.6 | 8.0 | 8.0 | ||
≥ 4 | 18 | 23.1 | 13.6 | 13.0 | 0.007 | |
Mother workse | NO | 39 | 55.7 | 10.4 | 9.0 | |
YES | 31 | 44.3 | 10.0 | 10.0 | 0.781 | |
Father workse | NO | 9 | 13.2 | 7.4 | 8.0 | |
YES | 59 | 86.8 | 10.7 | 10.0 | 0.160 | |
Workdays lost by caregivere | NO | 17 | 23.3 | 7.7 | 7.0 | |
YES | 56 | 76.7 | 11.2 | 10.0 | 0.033 |
*Independent-samples Mann–Whitney U test for two samples, and Kruskal–Wallis 1-way ANOVA for 3 or more samples, P values < 0.05 are in bold
aLI: lamellar ichthyosis, includes 2 patients with harlequin ichthyosis
bCIE: congenital ichthyosiform erythroderma
cPPK: palmoplantar keratoderma
dClinical severity score: mild-moderate 0–5, severe ≥ 6 signs and/or symptoms
eTotals may vary because of missing values