Table 2 Patient- and caregiver-reported complications experienced due to epidermolysis bullosa by type
Complication, n (%) | Patient-reported complications n = 62a | Caregiver-reported complications n = 92a | ||||||
|---|---|---|---|---|---|---|---|---|
Simplex n = 21 | Junctional n = 8 | Dominant dystrophic n = 14 | Recessive dystrophic n = 19 | Simplex n = 34 | Junctional n = 7 | Dominant dystrophic n = 17 | Recessive dystrophic n = 34 | |
Nail abnormalities | 15 (71.4) | 8 (100) | 13 (92.9) | 19 (100) | 23 (67.6) | 5 (71.4) | 14 (82.4) | 30 (88.2) |
Oral cavity problems | 7 (33.3) | 6 (75.0) | 12 (85.7) | 19 (100) | 12 (35.3) | 4 (57.1) | 12 (70.6) | 32 (94.1) |
Hand/foot contractures, webbing, severe scarring | 11 (52.4) | 6 (75.0) | 9 (64.3) | 16 (84.2) | 15 (44.1) | 1 (14.3) | 7 (41.2) | 30 (88.2) |
Constipation | 5 (23.8) | 5 (62.5) | 6 (42.9) | 13 (68.4) | 15 (44.1) | 3 (42.9) | 11 (64.7) | 25 (73.5) |
Dental problems | 7 (33.3) | 7 (87.5) | 8 (57.1) | 16 (84.2) | 5 (14.7) | 6 (85.7) | 8 (47.1) | 21 (61.8) |
Eye problems | 8 (38.1) | 2 (25.0) | 5 (35.7) | 11 (57.9) | 9 (26.5) | 6 (85.7) | 4 (23.5) | 25 (73.5) |
Anemia | 4 (19.0) | 5 (62.5) | 6 (42.9) | 16 (84.2) | 6 (17.6) | 2 (28.6) | 4 (23.5) | 24 (70.6) |
Problems with the scalp or hair loss | 5 (23.8) | 6 (75.0) | 7 (50.0) | 13 (68.4) | 13 (38.2) | 4 (57.1) | 2 (11.8) | 17 (50.0) |
Gastroesophageal reflux disorder | 4 (19.0) | 5 (62.5) | 8 (57.1) | 11 (57.9) | 7 (20.6) | 4 (57.1) | 3 (17.6) | 22 (64.7) |
Nutritional problems | 1 (4.8) | 3 (37.5) | 4 (28.6) | 13 (68.4) | 8 (23.5) | 4 (57.1) | 4 (23.5) | 25 (73.5) |
Esophageal strictures | 3 (14.3) | 3 (37.5) | 4 (28.6) | 19 (100) | 3 (8.8) | 1 (14.3) | 3 (17.6) | 24 (70.6) |
Depression requiring treatment | 6 (28.6) | 3 (37.5) | 9 (64.3) | 6 (31.6) | 2 (5.9) | 3 (42.9) | 3 (17.6) | 8 (23.5) |
Respiratory/breathing complications | 2 (9.5) | 0 | 4 (28.6) | 2 (10.5) | 6 (17.6) | 3 (42.9) | 4 (23.5) | 9 (26.5) |
Growth problems diagnosed by a physician | 0 | 1 (12.5) | 0 | 5 (26.3) | 8 (23.5) | 2 (28.6) | 1 (5.9) | 13 (38.2) |
Heart issues | 1 (4.8) | 0 | 2 (14.3) | 1 (5.3) | 2 (5.9) | 0 | 3 (17.6) | 8 (23.5) |
Lower urinary and genital abnormalities (eg, urethral stricture, difficulty urinating) | 2 (9.5) | 2 (25.0) | 4 (28.6) | 2 (10.5) | 2 (5.9) | 3 (42.9) | 0 | 2 (5.9) |
Delayed puberty | 0 | 5 (62.5) | 2 (14.3) | 6 (31.6) | 0 | 0 | 0 | 3 (8.8) |
Osteoporosis/bone fractures | 1 (4.8) | 2 (25.0) | 1 (7.1) | 2 (10.5) | 1 (2.9) | 0 | 1 (5.9) | 7 (20.6) |
Skin cancer (squamous cell carcinoma) | 4 (19.0) | 2 (25.0) | 1 (7.1) | 3 (15.8) | 1 (2.9) | 0 | 1 (5.9) | 0 |
Kidney problems | 1 (4.8) | 3 (37.5) | 3 (21.4) | 1 (5.3) | 1 (2.9) | 0 | 0 | 3 (8.8) |
Hearing loss | 0 | 1 (12.5) | 0 | 4 (21.1) | 1 (2.9) | 0 | 1 (5.9) | 1 (2.9) |
Otherb | 0 | 0 | 0 | 0 | 1 (2.9) | 0 | 1 (5.9) | 2 (5.9) |