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Summary

A standardized questionnaire was used to assess mobility, activity and pain in 140 randomly chosen children, who were representative of all major types and subtypes of inherited epidermolysis bullosa (EB). Pain status in these children was compared with 374 randomly selected adults with EB. The level of independence for each of six activities of daily living (ADL) (toileting; feeding; bathing; dressing; grooming; walking) was assessed in these EB children using conventional criteria for scoring. Whereas more than 90% of all EB simplex (EBS) and dominant dystrophic EB (DDEB) children were totally independent for each function (excluding walking), the frequency of similarly totally independent patients with junctional EB (JEB) and recessive dystrophic EB (RDEB) ranged from only 39% to 73%. No DDEB children and only 2% of EBS patients were totally dependent in their individual ADL, in comparison to 8–27% of JEB and 2–27% of RDEB children. Totally independent walking was reported in only 31%, 31%, 67%, and 24% of EBS, JEB, DDEB, and RDEB children, respectively. A daily level of EB-related pain was assessed in children by their parents using a linear scale of 0 (no pain) to 10 (unbearable pain). Whereas 14–19% of all children with EBS, JEB, and DDEB were graded with pain levels of more than 5, 32% of all RDEB children reportedly suffered this much pain. Increased frequencies of pain with scores more than 5 were most often noted in those patients having more clinically extensive or severe EB subtypes. These included JEB-Herlitz (20% vs. 14% in JEB-non-Herlitz) and RDEB-Hallopeau-Siemens (47% vs. 20% in all other RDEB subtypes). Only 5% of all RDEB children reportedly were pain-free, compared to 12–14% of those with EBS, JEB, and DDEB. Collectively, these data provide the first report of the specific impact different forms of EB have on daily living and coping with this genodermatosis.