Table II.
Summary of patient SCC data by epidermolysis bullosa subtype
| Total | RDEB-S | RDEB-Int | RDEB-Inv | RDEB-Pru | DDEB | JEB-Int | KEB | |
|---|---|---|---|---|---|---|---|---|
| Total patients, n | 44 | 31 | 2 | 1 | 1 | 5 | 3 | 1 |
| Sex, n (%) | ||||||||
| Male | 21 (46) | 16 | 1 | 0 | 0 | 2 | 2 | 0 |
| Female | 23 (52) | 15 | 1 | 1 | 1 | 3 | 1 | 1 |
| Ethnicity, n (%) | ||||||||
| Caucasian | 42 (95) | 29 | 2 | 1 | 1 | 5 | 3 | 1 |
| Asian | 2 (5) | 2 | 0 | 0 | 0 | 0 | 0 | 0 |
| Age diagnosis 1st SCC, median, years | 32.8 | 29.5 | 55.1 | 44.2 | 31.1 | 60.1 | 40.6 | 30.1 |
| Age diagnosis 1st SCC, mean, years | 32.8 | 30.5 | 55.1 | 44.2 | 31.1 | 62.5 | 49.4 | 30.1 |
| Range (years) | 13–89 | 13–52 | 42–68 | – | – | 47–89 | 33–74 | – |
| Number of primary SCCs, median | 2.5 | 3 | 1 | 1 | 10 | 2 | 2 | 5 |
| Number of primary SCCs, mean | 5 | 5.8 | 1 | 1 | 10 | 3 | 2.7 | 5 |
| Range number of primary SCCs | 1–44 | 1–44 | 1 | – | – | 1–7 | 1–5 | – |
| Metastatic disease (% by subtype), n (%) | 17 (39) | 16 (52%) | 0 | 0 | 0 | 0 | 1 (33%) | 0 |
| Deceased (% by subtype), n (%) | 25 (57) | 21 (68%) | 0 | 0 | 0 | 2 (40%) | 2 (67%) | 0 |
| Death related to SCC (% by subtype), n (%) | 22/25 (88) | 20/21 (95%) | 0 | 0 | 0 | 0 | 2/2 (100%) | 0 |
| Survival after 1st SCC, median, years | 2.1 | 2.4 | – | – | – | 1.6 | 0.7 | – |
| Survival after 1st SCC, mean, years | 3.4 | 3.8 | – | – | – | 1.6 | 0.7 | – |
| Survival after 1st SCC, range, years | 0.4–12.6 | 0.5–12.6 | – | – | – | 0.4–2.7 | 0.4–1 | – |
| Histological differentiation of 1st SCC | ||||||||
| In situ, n | 2 | 1 | 1 | 0 | 0 | 0 | 0 | 0 |
| Well differentiated, n | 21 | 11 | 1 | 1 | 1 | 4 | 2 | 1 |
| Moderately differentiated, n | 16 | 15 | 0 | 0 | 0 | 1 | 0 | 0 |
| Poorly differentiated, n | 5 | 4 | 0 | 0 | 0 | 1 | 1 | 0 |
RDEB-S: recessive dystrophic epidermolysis bullosa severe; RDEB-Int: recessive dystrophic epidermolysis bullosa intermediate; RDEB-Inv: recessive dystrophic epidermolysis bullosa inversa; RDEB-Pru: recessive dystrophic epidermolysis bullosa pruriginosa; DDEB: dominant dystrophic epidermolysis bullosa; JEB-Int: junctional epidermolysis bullosa intermediate; KEB: Kindler epidermolysis bullosa; SCC: squamous cell carcinoma.