Itch and pain reported by individuals with recessive dystrophic epidermolysis bullosa (RDEB): Findings of the pebles study.

Introduction &objectives: Introduction: Itch and pain are acknowledged problems for individuals with RDEB. PEBLES is a prospective register study to record detailed information about what happens to an individual with RDEB and how it affects them over time, including itch and pain. To report preliminary findings regarding itch and pain reported by individuals with RDEB. Materials &methods: Methods: Individuals recruited to PEBLES were reviewed annually for those 10 years and older and 6-monthly for those under 10 years. All participants reported background and procedural pain (dressing changes) on a 10cm visual analogue scale (VAS) and indicated the number of nights their sleep was disturbed by pain. Participants aged 8 years and above also completed the Leuven Itch Scale (version 1.0). The study was ethically approved by the UK Research Ethics Committee and Health Research Authority. Result(s): Data regarding itch and pain at initial review were available for 38 adults (79%) and 10 children (21%). Of these, 31 (65%) completed four reviews spanning 2-4 years. Participants with RDEB generalised severe (RDEB-GS) reported the greatest number of nights sleep disturbed by pain: only 19% had undisturbed sleep compared to 39% of individuals with RDEB generalised intermediate (RDEB-GI) and 29% with RDEB-other. Participants in all subtypes reported significant background pain (median 4.3, IQR 2.9,6.0) with greater procedural pain (median 6.0, IQR 4.0,7.6); background and procedural pain levels were greatest for participants with RDEB-GS. Individuals with RDEB-GS (n=36) experienced more frequent itch, greater severity and distress, but shortest duration. More than half of all individuals reported consequences of itching such as skin damage, disturbed routine, difficulty falling asleep, being woken up by itchd mood and loss of concentration. Reduced quality of life was a common consequence of itch in RDEB-GS not other subtypes. Nearly half (44%) were not using treatment for it