Table 2 Disease burden among respondents with epidermolysis bullosa simplex (EBS), by age
All, No. (%) (n = 214) | Age 0–9, No. (%) (n = 33) | Age 10–17, No. (%) (n = 26) | Age 18 + , No. (%) (n = 155) | P Value | |
|---|---|---|---|---|---|
Clinical characteristics | |||||
EBS subtype | 0.03* | ||||
Localized | 94 (43.9) | 9 (27.3) | 7 (26.9) | 78 (50.3) | |
Intermediate | 9 (4.2) | 2 (6.1) | 1 (3.8) | 6 (3.9) | |
Severe | 26 (12.1) | 7 (21.2) | 6 (23.1) | 13 (8.4) | |
Other or unsure which EBS subtypea | 85 (39.7) | 15 (45.5) | 12 (46.2) | 58 (37.4) | |
Self-reported disease severity | 0.001* | ||||
Mild | 82 (38.3) | 14 (42.4) | 11 (42.3) | 57 (36.8) | |
Moderate | 100 (46.7) | 8 (24.2) | 10 (38.5) | 82 (52.9) | |
Severe | 21 (9.8) | 8 (24.2) | 5 (19.2) | 8 (5.2) | |
Not answered | 11 (5.1) | 3 (9.1) | 0 (0.0) | 8 (5.2) | |
Diagnostic confirmation with genetic testing or skin biopsy | 63 (29.4) | 16 (48.5) | 9 (34.6) | 35 (22.6) | < 0.001* |
Mean worst pain in past 12 months, ± SD | 6.8 ± 2.8 | 6.5 ± 2.8 | 6.9 ± 3.3 | 6.8 ± 2.7 | 0.64 |
Mean QOLEB, ± SDb | 14.7 ± 7.5 | 15.3 ± 10.3 | 16.6 ± 7.5 | 14.3 ± 7.2 | 0.75 |
Body mass indexc | < 0.001* | ||||
Underweight | 12 (5.6) | 1 (3.0) | 4 (15.4) | 7 (4.5) | |
Healthy weight | 63 (29.4) | 9 (27.3) | 11 (42.3) | 43 (27.7) | |
Overweight/obese | 81 (37.9) | 2 (6.1) | 0 (0.0) | 79 (51.0) | |
Not answered | 58 (27.1) | 21 (63.6) | 11 (42.3) | 26 (16.8) | |
Clinical manifestations | |||||
Blisters | 199 (93.0) | 29 (87.9) | 26 (100.0) | 144 (92.9) | 0.21 |
Pain | 158 (73.8) | 22 (66.7) | 23 (88.5) | 113 (72.9) | 0.15 |
Itch | 118 (55.1) | 17 (51.5) | 19 (73.1) | 82 (52.9) | 0.15 |
Difficulty walking | 94 (43.9) | 8 (24.2) | 12 (46.2) | 74 (47.7) | 0.04* |
Constipation | 91 (42.5) | 15 (45.5) | 12 (46.2) | 64 (41.3) | 0.84 |
Nail problems | 84 (39.3) | 15 (45.5) | 14 (53.8) | 55 (35.5) | 0.15 |
Infections | 74 (34.6) | 11 (33.3) | 13 (50.0) | 50 (32.3) | 0.24 |
Dental caries | 54 (25.2) | 5 (15.2) | 11 (42.3) | 38 (24.5) | 0.07 |
Ophthalmic problems | 34 (15.9) | 1 (3.0) | 3 (11.5) | 30 (19.4) | 0.04* |
Milia | 33 (15.4) | 7 (21.2) | 5 (19.2) | 21 (13.5) | 0.40 |
Difficulty swallowing | 32 (15.0) | 7 (21.2) | 4 (15.4) | 21 (13.5) | 0.45 |
Anemia | 27 (12.6) | 3 (9.1) | 3 (11.5) | 21 (13.5) | 0.94 |
Difficulty sleeping | 25 (11.7) | 2 (6.1) | 3 (11.5) | 20 (12.9) | 0.61 |
Genitourinary problems | 17 (7.9) | 1 (3.0) | 3 (11.5) | 13 (8.4) | 0.46 |
Hair problems | 17 (7.9) | 3 (9.1) | 1 (3.8) | 13 (8.4) | 0.84 |
Failure to thrive | 12 (5.6) | 3 (9.1) | 4 (15.4) | 5 (3.2) | 0.02* |
Gastrostomy tube | 9 (4.2) | 5 (15.2) | 3 (11.5) | 1 (0.6) | < 0.001* |
Wound characteristics | |||||
Presence of small wounds (< 2.5 cm)d | 165 (77.1) | 28 (84.8) | 18 (69.2) | 119 (76.8) | 0.38 |
Presence of medium wounds (2.5–7.5 cm)d | 107 (50.0) | 18 (54.5) | 13 (50.0) | 76 (49.0) | 0.86 |
Presence of large wounds (> 7.5 cm)d | 62 (29.0) | 8 (24.2) | 11 (42.3) | 43 (27.7) | 0.28 |
Presence of chronic woundse | 126 (58.9) | 23 (69.7) | 18 (69.2) | 85 (54.8) | 0.15 |
Anatomic location of chronic woundse | |||||
Head and neck | 28 (13.1) | 7 (21.2) | 2 (7.7) | 19 (12.3) | 0.30 |
Upper extremities | 37 (17.3) | 9 (27.3) | 5 (19.2) | 23 (14.8) | 0.21 |
Trunk and lower back | 8 (3.7) | 3 (9.1) | 2 (7.7) | 3 (1.9) | 0.05 |
Buttocks and genitals | 12 (5.6) | 5 (15.2) | 2 (7.7) | 5 (3.2) | 0.02* |
Lower extremities | 116 (54.2) | 22 (66.7) | 17 (65.4) | 17 (11.0) | 0.11 |
Presence of recurrent woundsf | 191 (89.3) | 31 (93.9) | 22 (84.6) | 138 (89.0) | 0.49 |
Anatomic location of recurrent woundsf | |||||
Head and neck | 47 (22.0) | 12 (36.4) | 8 (30.8) | 27 (17.4) | 0.03* |
Upper extremities | 88 (41.1) | 16 (48.5) | 10 (38.5) | 62 (40.0) | 0.68 |
Trunk and lower back | 19 (8.9) | 5 (15.2) | 2 (7.7) | 12 (7.7) | 0.36 |
Buttocks and genitals | 31 (14.5) | 8 (24.2) | 3 (11.5) | 20 (12.9) | 0.24 |
Lower extremities | 183 (85.5) | 30 (90.9) | 22 (84.6) | 131 (84.5) | 0.71 |
Number of dressing changes per week | 0.16 | ||||
0 to 3 changes/week | 56 (26.2) | 11 (33.3) | 5 (19.2) | 40 (25.8) | |
4 to 9 changes/week | 80 (37.4) | 14 (42.4) | 7 (26.9) | 59 (38.1) | |
10 + changes/week | 11 (5.1) | 1 (3.0) | 4 (15.4) | 6 (3.9) | |
Not answered | 67 (31.3) | 7 (21.2) | 10 (38.5) | 50 (32.3) | |
Medication Use | |||||
Non-opiate analgesics (routine use)g | 107 (50.0) | 19 (57.6) | 14 (53.8) | 74 (47.7) | 0.55 |
Topical antibioticsh | 92 (43.0) | 22 (66.7) | 18 (69.2) | 52 (33.5) | < 0.001* |
Non-opiate analgesics (dressing changes)g | 61 (28.5) | 15 (45.5) | 8 (30.8) | 38 (24.5) | 0.05 |
Antihistaminesi | 45 (21.0) | 12 (36.4) | 5 (19.2) | 28 (18.1) | 0.08 |
Laxativesj | 34 (15.9) | 5 (15.2) | 6 (23.1) | 23 (14.8) | 0.57 |
Opiates (routine use)k | 26 (12.1) | 7 (21.2) | 3 (11.5) | 16 (10.3) | 0.24 |
Psychiatric medicationsl | 24 (11.2) | 0 (0.0) | 4 (15.4) | 20 (12.9) | 0.05* |
Topicals steroidsm | 16 (7.5) | 4 (12.1) | 0 (0.0) | 12 (7.7) | 0.23 |
Opiates (dressing changes)k | 12 (5.6) | 3 (9.1) | 0 (0.0) | 9 (5.8) | 0.39 |
Systemic steroidsn | 6 (2.8) | 0 (0.0) | 1 (3.8) | 5 (3.2) | 0.66 |