Quality of life and economic burden in recessive dystrophic epidermolysis bullosa

Abstract

Background: Recessive dystrophic epidermolysis bullosa (RDEB) patients present blisters and erosions since birth, which cause pain, pruritus and various complications. RDEB affects quality of life (QoL) of patients in physical, emotional and social aspects. Besides, interminable dressings and supportive therapies imposes a significant economic burden on the EB family.Objective: To assess the quality of life and economic burden in RDEB patients.Methods: Sixteen RDEB patients were invited asking for their QoL and economic burden. Patients answered questionnaire consisting of visual analogue scale (VAS) on pain and pruritus, Sindex-29, Quality of Life in EB questionnaire (QOLEB) and their economic burden due to EB. Results: Thirteen RDEB patients completed the questionnaire. Female patients presented higher VAS, QOLEB and total Skindex-29 scores than male patients. RDEB patients showed severe level of pruritus, which was more intolerable than pain. Mean VAS score on pain in RDEB was higher than oral lichen planus and postherpetic neuralgia, VAS score on pruritus was similar to chronic urticaria, atopic dermatitis and prurigo nodularis. Compared with other dermatologic conditions, RDEB patient were profoundly affected in all three scales of skindex-29. Mean “medical costs” in a month was 257.54 United States Dollar (USD) (±169.39) and mean “dressing costs” was 358.41 USD (±312.55), which was negatively related to patient age. QoL and economic burden were not significantly different among RDEB subtypes, BSA involved, hospitalization days and number of accompanying complications.Conclusion: RDEB had profound impact on QoL and economic burden. Female patients and patient with more severe perceived disease severity presented worse QoL. Compared with other dermatologic diseases, RDEB showed severe symptoms and their QoL was seriously impaired. Most patients had economic burden, especially on preparing dressing materials, younger patients had more economic burden.