Table 1.
Frequency of ocular findings in the National EB Registry study population by EB subtype (%)
| EB Simplex | Junctional EB | Dystrophic EB | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Manifestation | Localised (N = 1092) | G-S (N = 113) | G-I (N-96) | Ogna (N = 379) | G-S (N = 40) | G-I (N = 190) | DDEB G (N = 424 | RDEB G-S (N = 139) | RDEB G-I (N = 265) | RDEB Inversa (N = 17) |
| Corneal erosions/blisters | 0.92 | 6.19 | 3.13 | 2.64 | 47.50 | 25.26 | 2.12 | 74.10 | 32.45 | 35.29 |
| Corneal scarring | 0.27 | 0.00 | 3.16 | 0.53 | 26.83 | 13.37 | 0.95 | 50.00 | 16.92 | 29.41 |
| Symblepharon | 0.00 | 0.00 | 0.00 | 0.00 | 4.76 | 2.11 | 0.00 | 10.07 | 1.89 | 11.76 |
| Blepharitis | 0.37 | 0.88 | 2.08 | 0.26 | 7.14 | 6.32 | 0.71 | 17.52 | 6.46 | 17.65 |
| Ectropion | 0.00 | 0.00 | 0.00 | 0.00 | 14.29 | 2.11 | 0.00 | 7.19 | 1.90 | 0.00 |
| Lacrimal duct obstruction | 1.19 | 2.65 | 1.04 | 1.85 | 2.38 | 4.23 | 1.65 | 5.80 | 5.30 | 11.76 |
| Impaired vision | 13.17 | 13.27 | 15.63 | 16.14 | 16.67 | 13.68 | 17.18 | 38.13 | 21.89 | 41.18 |
| Blindness | 0.82 | 1.77 | 0.00 | 0.53 | 0.00 | 1.58 | 0.94 | 6.47 | 1.14 | 0.00 |
Abbreviations: G-S = generalised-severe; G-I = generalised-intermediate; G = generalised
Source: Fine JD, Johnson LB, Weiner M, Stein A, Cash S, Deleoz J, et al. Eye involvement in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry. Am J Ophthalmol. 2004;138:254–262. (Permission for reuse obtained under RightsLink)