Table 4.
Comparison of the studied variables in inherited epidermolysis bullosa patients according to their BEBS score and controls.
| Parameter | Total IEB Cases n = 42 | EB Patients with Low-Medium BEBS Score n = 28 | EB Patients with High BEBS Score n = 14 | p-Value | Corrected p-Value * | Controls n = 38 | p-Value | Corrected p-Value * |
|---|---|---|---|---|---|---|---|---|
| Anti-skin antibodies, U/mL | ||||||||
| DSG1 | 3.83 (0.18–38.83) | 2.6 (0.18–20.2) | 6.22 (2.18–38.83) | 0.002 | 0.008 | 2.12 (0–15.09) | 0.291 | 0.312 |
| DSG3 | 3.72 (0–40.4) | 2.84 (0–35.13) | 5.45 (0.94–40.4) | 0.004 | 0.012 | 1.58 (0.08–8.38) | 0.02 | 0.033 |
| BP180 | 7.1 (0.5–118.8) | 5.9 (0.5–26.3) | 23.3 (3.3–118.8) | 0.001 | 0.005 | 1.82 (0–34.41) | 0.007 | 0.018 |
| BP230 | 4.75 (0.5–66) | 3.65 (0.5–15.6) | 13.7 (1.97–66) | <0.001 | <0.001 | 1.68 (0.22–26.74) | 0.012 | 0.022 |
| COL VII | 1.92 (0–41.05) | 1.08 (0–8.2) | 7.39 (0.72–41.05) | <0.001 | <0.001 | 0.26 (0–3.99) | <0.001 | <0.001 |
| Cytokines, pg/mL | ||||||||
| IL-1β | 16.62 (1.16–192.67) | 7.71 (1.16–180.69) | 46.78 (3.78–192.67) | 0.038 | 0.081 | 2.75 (0.88–189.66) | 0.005 | 0.015 |
| IL-2 | 111.23 (18.76–3992.66) | 96.42 (18.76–3992.66) | 273.24 (34.86–3475.02) | 0.238 | 0.397 | 31.61 (8.01–2400) | 0.001 | 0.004 |
| IL-4 | 0.96 (0.6–4.5) | 0.96 (0.6–4.5) | 0.93 (0.79–3.26) | 0.727 | 0.875 | 0.94 (0.6–2.07) | 0.231 | 0.289 |
| IL-6 | 33.44 (5.32–1000) | 16.99 (5.32–1000) | 50.77 (17.23–858.09) | 0.009 | 0.022 | 8.54 (4.46–1000) | 0.001 | 0.004 |
| IL-8 | 44.3 (4.4–449) | 47.4 (4.4–449) | 42.18 (21.97–124.24) | 0.927 | 0.927 | 27.96 (12.96–939.06) | 0.282 | 0.312 |
| IL-10 | 32.98 (8–250) | 44.75 (8–250) | 30.95 (17–187.6) | 0.817 | 0.875 | 21.12 (5.86–250) | 0.028 | 0.038 |
| IL-12 | 1.52 (1.14–9.21) | 1.56 (1.14–9.21) | 1.4 (1.22–2.34) | 0.157 | 0.294 | 1.36 (1–7.29) | 0.026 | 0.038 |
| TNF-α | 175.12 (137.66–4800) | 167.75 (137.66–1364.65) | 198.13 (142.5–4800) | 0.497 | 0.683 | 176.1 (128.17–1351.09) | 0.657 | 0.657 |
| TNF-β | 1737.07 (1101.8–36,000) | 1737.07 (1101.8–36,000) | 1717.87 (1149.51–36,000) | 0.787 | 0.875 | 1267.11 (802.84–3764.79) | <0.001 | <0.001 |
| IFN-γ | 330.16 (79.18–5000) | 303.38 (79.18–5000) | 387.95 (110.6–4607.18) | 0.501 | 0.683 | 176.34 (73.56–4910) | 0.01 | 0.021 |
BEBS: Birmingham Epidermolysis Bullosa Severity score; BP180: antibodies against bullous pemphigoid 180; BP230: antibodies against bullous pemphigoid 230; COL7: type VII collagen; DSG1: antibodies against desmoglein 1; DSG3: antibodies against desmoglein 3; EB: inherited epidermolysis bullosa; IFN: interferon; IL: interleukin; TNF: tumor necrosis factor. Median values (range in parenthesis). * p-Values were adjusted for multiple testing using the false discovery rate method (with the Benjamini-Hochberg procedure). Controls were compared with EB patients with a low-to-medium BEBS score.