Articles
18 May 2023
Vol. 15 No. 4 (2023)
https://doi.org/10.4081/dr.2023.9700

Measuring the impact of pruritus in patients with epidermolysis bullosa: evaluation with an itch-specific instrument

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Epidermolysis bullosa, pruritus, itch, genodermatoses
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Authors

Ashjan Alheggi
aialheggi@imamu.edu.sa
https://orcid.org/0000-0002-4869-4684
Department of Dermatology, College of Medicine, Imam Mohammad Ibn Saud Islamic University, Riyadh, Saudi Arabia.
Raneem Alnutaifi
https://orcid.org/0009-0004-7443-2938
College of Medicine, Imam Mohammad Ibn Saud Islamic University, Riyadh, Saudi Arabia.
Manal Alkhonezan
https://orcid.org/0009-0002-4594-2619
College of Medicine, Imam Mohammad Ibn Saud Islamic University, Riyadh, Saudi Arabia.
Norah Almudawi
College of Medicine, Imam Mohammad Ibn Saud Islamic University, Riyadh, Saudi Arabia.
Renad Alsuhaibani
College of Medicine, Imam Mohammad Ibn Saud Islamic University, Riyadh, Saudi Arabia.
Philip Moons
KU Leuven Department of Public Health and Primary Care, KU Leuven, Belgium.
Turki Aljuhani
https://orcid.org/0000-0001-9328-2075
Department of Dermatology, King Fahad University Hospital, Khober, Saudi Arabia.

Pruritus is one of the most debilitating symptoms for patients with epidermolysis bullosa (EB). This study aimed to assess the burden of itch and to address its dimensions across patients with EB. Forty-six patients with EB were recruited from the Saudi EB registry to participate. All participants completed the Leuven Itch Scale. The sample included 5 patients with EB simplex (EBS), 3 with junctional EB (JEB), 34 with dystrophic EB (DEB), and 4 patients had unknown type. Overall, 97.8% patients reported itch. In patients with itch, 73.3% reported that it was often or always present, longer than 2h Itch episodes was reported by JEB (66.7%) and recessive DEB (RDEB) (3.2%). Itch, in all its dimensions, was worst in patients with JEB and DEB than EBS. Itch occurred mostly in a hot environment (80%), when sweating (71.1%), in healing wounds (40%), and during dressing change (35.6%) whereas cold environment resulted in itch in only (2.2%). The burden of pruritus increased with increasing age. This study highlights a challenging area in EB care with a need for specific treatments.

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Crossref
Scopus
Google Scholar
Europe PMC
Has C, Bauer JW, Bodemer C, et al. Consensus reclassification of inherited epidermolysis bullosa and other disorders with skin fragility. Br J Dermatol 2020;183:614-27.
Bardhan A, Bruckner-Tuderman L, Chapple ILC, et al. Epidermolysis bullosa. Nat Rev Dis Primers 2020;6:78.
van Scheppingen C, Lettinga AT, Duipmans JC, et al. Main problems experienced by children with epidermolysis bullosa: a qualitative study with semi-structured interviews. Acta Derm Venereol 2008;88:143-50.
Snauwaert JJL, Yuen WY, Jonkman MF, et al. Burden of itch in epidermolysis bullosa. Br J Dermatol 2014;171:73-8.
Danial C, Adeduntan R, Gorell ES, et al. Prevalence and characterization of pruritus in epidermolysis bullosa. Pediatr Dermatol 2015;32:53-9.
Jeon IK, On HR, Kim SC. Quality of life and economic burden in recessive dystrophic epidermolysis bullosa. Ann Dermatol 2016;28:6-14.
Dalgard FJ, Svensson Å, Halvorsen JA, et al. Itch and mental health in dermatological patients across Europe: A cross-sectional study in 13 countries. J Invest Dermatol 2020;140:568-73.
Haest C, Casaer MP, Daems A, et al. Measurement of itching: validation of the Leuven Itch Scale. Burns 2011;37:939-50.
De Palma AM, Mazereeuw-Hautier J, Giehl K, et al. Burden of itch in ichthyosis: a multicentre study in 94 patients. J Eur Acad Dermatol Venereol 2019;33:2095-100.
Papanikolaou M, Onoufriadis A, Mellerio JE, et al. Prevalence, pathophysiology and management of itch in epidermolysis bullosa. Br J Dermatol 2021;184:816-25.
Nguyen HH, Shinkuma S, Hayashi R, et al. New insight of itch mediators and proinflammatory cytokines in epidermolysis bullosa. J Cutan Immunol Allergy 2022;5:78-87.
Goldschneider KR, Lucky AW. Pain management in epidermolysis bullosa. Dermatol Clin 2010;28:273-82.
Davila-Seijo P, Hernández-Martín A, Morcillo-Makow E, et al. Prioritization of therapy uncertainties in dystrophic epidermolysis bullosa: where should research direct to? an example of priority setting partnership in very rare disorders. Orphanet J Rare Dis 2013;8:61.
Chiou AS, Choi S, Barriga M, et al. Phase 2 trial of a neurokinin-1 receptor antagonist for the treatment of chronic itch in patients with epidermolysis bullosa: a randomized clinical trial. J Am Acad Dermatol 2020;82:1415-21.
Rashidghamat E, Kadiyirire T, Ayis S, et al. Phase I/II open-label trial of intravenous allogeneic mesenchymal stromal cell therapy in adults with recessive dystrophic epidermolysis bullosa. J Am Acad Dermatol 2020;83:447-54.
Castela E, Tulic MK, Rozières A, et al. Epidermolysis bullosa simplex generalized severe induces a T helper 17 response and is improved by apremilast treatment. Br J Dermatol 2019;180:357-64.
Shehadeh W, Sarig O, Bar J, et al. Treatment of epidermolysis bullosa pruriginosa-associated pruritus with dupilumab. Br J Dermatol 2020;182:1495-7.
Philip Moons, KU Leuven Department of Public Health and Primary Care, KU Leuven

Institute of Health and Care Sciences, University of Gothenburg, Sweden

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Measuring the impact of pruritus in patients with epidermolysis bullosa: evaluation with an itch-specific instrument. (2023). Dermatology Reports, 15(4). https://doi.org/10.4081/dr.2023.9700
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