Prevalence and Characterization of Pruritus in Epidermolysis Bullosa

Abstract

Background Qualitative data suggest that pruritus is a burdensome symptom in patients with epidermolysis bullosa (EB), but the prevalence of pruritus in children and adults with EB, as well as factors that contribute to pruritus are unknown. Objective To quantitatively identify and to characterize pruritus experienced by EB patients using a comprehensive online questionnaire. Methods A questionnaire was developed to evaluate pruritus in all ages and types of EB. Questions that characterize pruritus were included, and factors that aggravate symptoms were investigated. Patients from seven North American EB centers were invited to participate. Results A total of 146 out of 216 questionnaires were completed (response rate=68%) (73 males, 73 females; median age, 20.0). Using a 5-point Likert scale (1=never, 2=rarely, 3=sometimes, 4=often, 5=always), itchiness was the most bothersome EB complication (mean=3.3). The average daily frequency of pruritus increased with self-reported EB severity. Pruritus was most frequent at bedtime (mean=3.8) and interfered with sleep. Factors that aggravated pruritus included healing wounds, dry skin, infected wounds, stress, heat, dryness, and humidity. Conclusions Pruritus is common in EB patients and can be very bothersome. Future studies will need to investigate the most effective treatments given to EB patients for pruritus.

TLR3 in Chronic Human Itch: A Keratinocyte-Associated Mechanism of Peripheral Itch Sensitization

“…Itch in this setting has very significant implications for patients, leading to further significant injury and complications from scratching (Danial et al 2015). Chronic venous ulceration is also associated with significant itch, which can impact upon healing and add to patient morbidity (Paul et al 2011).…”

The Effects of Cool Dialysate on Pruritus Status During Hemodialysis of Patients With Chronic Renal Failure: A Controlled Randomized Clinical Trial

“…Previous research showed that heat aggravated pruritus, so individuals who are prone to pruritus are recommended to avoid heat (9). The process of hemodialysis (a dialysis solution with a standard temperature of 37°C) causes vasodilation of the blood vessels in the skin, which increases the body temperature (10).…”

Evaluation of Treatments for Pruritus in Epidermolysis Bullosa

“…The exposure of open wounds to water while bathing can be painful and can increase pruritus in some individuals with EB . It is unclear whether this increase in pruritus is secondary to stress or pain.…”