Table 1.
Demographic and treatment information for patients with disorders of keratinization administered systemic retinoid therapy.
| Characteristics | Total (n = 127) | Treatment groups a | ||
|---|---|---|---|---|
| Acitretin (n = 107) | Isotretinoin (n = 40) | Alitretinoin (n = 6) | ||
| Female sex, n (%) | 66 (52.0) | 48 (44.9) | 35 (87.5) | 4 (66.7) |
| Diseases treated | ||||
| Ichthyosis | 51 (38.6) | 42 (39.3) | 18 (45.0) | 3 (50.0) |
| Palmoplantar keratoderma | 32 (25.2) | 28 (26.2) | 7 (17.5) | 3 (50.0) |
| Darier disease | 27 (21.3) | 24 (22.4) | 8 (20.0) | 0 (0) |
| Pachyonychia congenita | 5 (3.9) | 5 (4.7) | 0 (0) | 0 (0) |
| Hailey–Hailey disease | 4 (3.1) | 3 (2.8) | 1 (2.5) | 0 (0) |
| Other b | 8 (6.3) | 5 (4.7) | 6 (15.0) | 0 (0) |
| Age at commencement, years c , d | 31.9 ± 17.1 | 32.1 ± 17.3 | 27 (19;33) | 35 (29; 38) |
| Total cumulative dose, mg, median (Q1, Q3) d | – |
45 734.5 (16 425; 109 500) |
21 900 (7300; 73 000) |
27 375 (17 793.8; 32 850.0) |
| Daily dose, mg/kg/day, mean ± SD | – | 0.33 ± 0.22 | 0.36 ± 0.22 | NA |
| Time on treatment, years c , d | 11.2 ± 110.0 | 10.3 ± 110.1 | 2.9 (1; 7) | 3 (1.9; 5.3) |
| Ossification, n (%) | 3 (2.4) | 1 (0.9) | 3 (7.9) | 0 (0) |
NA, not applicable; Q, quartile.
a
Treatment groups were not mutually exclusive.
b
Included acrokeratoelastoidosis, erythrokeratodermia variabilis, Papillon–Lefèvre syndrome, keratosis follicularis spinulosa keratosis, follicularis spinulosa decalvans, keratosis lichenoides chronica and lipoid proteinosis.
c
Mean ± SD or median (Q1, Q3).
d
Median (Q1, Q3) were used for data that were not normally distributed.