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Open Access 03-01-2024 | Epidermolysis Bullosa | Case Series

Oleogel-S10 in Dystrophic Epidermolysis Bullosa: A Case Series Evaluating the Impact on Wound Burden Over Two Years

Authors: Mauricio Torres Pradilla, Erick Álvarez, Mónica Novoa, Ivonne Lozano, Maribel Trujillo

Published in: Advances in Therapy | Issue 2/2024

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Abstract

Epidermolysis bullosa (EB) is a group of rare, difficult-to-treat, inherited multisystem diseases affecting epithelial integrity. Impaired wound healing is central and can lead to serious clinical complications, deformities, and symptoms with a devastating impact on quality of life (QoL). Dressing changes and wound care are central to the management of EB. Recently Oleogel-S10 (also known as birch bark extract or birch triterpenes) was approved in Europe and the UK for treating EB wounds. This approval was based on data from the EASE phase 3 study, which demonstrated Oleogel-S10 accelerated wound healing, reduced total wound burden, and decreased the frequency of dressing changes in patients with EB. A retrospective analysis of medical records was conducted for up to 24 months in 13 patients with EB treated with Oleogel-S10 through an early access programme in Colombia. Effectiveness was assessed by measuring body surface area percentage (BSAP) and total body wound burden (EBDASI). Tolerability and safety were monitored throughout. This is the first report to evaluate the effectiveness of Oleogel-S10 in clinical practice. The results showed a reduction in percentage of BSA affected, from a mean of 27.3% at baseline to 10.4% at 24-month follow-up, despite treatment interruptions. A reduction in EBDASI skin activity score of − 16.2 (24 months) together with a reduced skin damage index score of − 15.4 (18 months) was also observed. Physicians, patients, and caregivers perceived faster wound closure. Adherence with therapy by patients was good, and patients expressed satisfaction with treatment and reported improvements in self-esteem, productivity, and social interaction. Oleogel-S10 was well tolerated; however, two patients reported worsening wounds related to gauze adherence. Two deaths during treatment interruption were reported and was not considered related to Oleogel-S10. This study supports the effectiveness of Oleogel-S10 in a real-world scenario in a country with scarce resources for the treatment of EB.
Literature
1.
2.
go back to reference Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):367–84.CrossRefPubMed Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol. 2009;61(3):367–84.CrossRefPubMed
3.
go back to reference Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II Other organs. J Am Acad Dermatol. 2009;61(3):387–402.CrossRefPubMed Fine JD, Mellerio JE. Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part II Other organs. J Am Acad Dermatol. 2009;61(3):387–402.CrossRefPubMed
4.
go back to reference Togo CCG, Zidorio APC, Gonçalves VSS, Hubbard L, de Carvalho KMB, Dutra ES. Quality of life in people with epidermolysis bullosa: a systematic review. Qual Life Res. 2021;29(7):1731–45.CrossRef Togo CCG, Zidorio APC, Gonçalves VSS, Hubbard L, de Carvalho KMB, Dutra ES. Quality of life in people with epidermolysis bullosa: a systematic review. Qual Life Res. 2021;29(7):1731–45.CrossRef
7.
go back to reference Laszczyk M, Jäger S, Simon-Haarhaus B, Scheffler A, Schempp CM. Physical, chemical and pharmacological characterization of a new oleogel-forming triterpene extract from the outer bark of birch (betulae cortex). Planta Med. 2006;72(15):1389–95.CrossRefPubMed Laszczyk M, Jäger S, Simon-Haarhaus B, Scheffler A, Schempp CM. Physical, chemical and pharmacological characterization of a new oleogel-forming triterpene extract from the outer bark of birch (betulae cortex). Planta Med. 2006;72(15):1389–95.CrossRefPubMed
8.
go back to reference Schwieger-Briel A, Kiritsi D, Schempp C, Has C, Schumann H. Betulin-based oleogel to improve wound healing in dystrophic epidermolysis bullosa: a prospective controlled proof-of-concept study. Dermatol Res Pract. 2017;2017:5068969.CrossRefPubMedPubMedCentral Schwieger-Briel A, Kiritsi D, Schempp C, Has C, Schumann H. Betulin-based oleogel to improve wound healing in dystrophic epidermolysis bullosa: a prospective controlled proof-of-concept study. Dermatol Res Pract. 2017;2017:5068969.CrossRefPubMedPubMedCentral
9.
go back to reference Schwieger-Briel A, Ott H, Kiritsi D, Laszczyk-Lauer M, Bodemer C. Mechanism of Oleogel-S10: a triterpene preparation for the treatment of epidermolysis bullosa. Dermatol Ther. 2019;32(4):12983. Schwieger-Briel A, Ott H, Kiritsi D, Laszczyk-Lauer M, Bodemer C. Mechanism of Oleogel-S10: a triterpene preparation for the treatment of epidermolysis bullosa. Dermatol Ther. 2019;32(4):12983.
10.
go back to reference Kern JS, Sprecher E, Fernandez MF, et al. Efficacy and safety of Oleogel-S10 (birch triterpenes) for epidermolysis bullosa: results from the phase III randomized double-blind phase of the EASE study. Br J Dermatol. 2023;188(1):12–21.CrossRefPubMed Kern JS, Sprecher E, Fernandez MF, et al. Efficacy and safety of Oleogel-S10 (birch triterpenes) for epidermolysis bullosa: results from the phase III randomized double-blind phase of the EASE study. Br J Dermatol. 2023;188(1):12–21.CrossRefPubMed
11.
go back to reference Jain SV, Harris AG, Su JC, et al. The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI): grading disease severity and assessing responsiveness to clinical change in epidermolysis bullosa. J Eur Acad Dermatol Venereol. 2017;31:692–8.CrossRefPubMed Jain SV, Harris AG, Su JC, et al. The Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI): grading disease severity and assessing responsiveness to clinical change in epidermolysis bullosa. J Eur Acad Dermatol Venereol. 2017;31:692–8.CrossRefPubMed
12.
go back to reference Loh C, Kim J, Su J, et al. Development, reliability, and validity of a novel Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI). J Am Acad Dermatol. 2014;70(1):89–97.CrossRefPubMed Loh C, Kim J, Su J, et al. Development, reliability, and validity of a novel Epidermolysis Bullosa Disease Activity and Scarring Index (EBDASI). J Am Acad Dermatol. 2014;70(1):89–97.CrossRefPubMed
13.
go back to reference Mellerio JE, Kiritsi D, Marinkovich MP, et al. Mapping the burden of severe forms of epidermolysis bullosa—implications for patient management. J Am Acad Dermatol Int. 2023;11:224–32. Mellerio JE, Kiritsi D, Marinkovich MP, et al. Mapping the burden of severe forms of epidermolysis bullosa—implications for patient management. J Am Acad Dermatol Int. 2023;11:224–32.
14.
go back to reference Bruckner AL, Losow M, Wisk J, et al. The challenges of living with and managing epidermolysis bullosa: insights from patients and caregivers. Orphanet J Rare Dis. 2023;15(1):1.CrossRef Bruckner AL, Losow M, Wisk J, et al. The challenges of living with and managing epidermolysis bullosa: insights from patients and caregivers. Orphanet J Rare Dis. 2023;15(1):1.CrossRef
15.
go back to reference Murrell D, Bruckner AL, Sprecher E, et al. BG04 Oleogel-S10 (birch triterpenes) in the treatment of epidermolysis bullosa wounds: 24-month efficacy and safety data from the EASE study. Br J Dermatol. 2023;188(Suppl 4):ljad113-144. Murrell D, Bruckner AL, Sprecher E, et al. BG04 Oleogel-S10 (birch triterpenes) in the treatment of epidermolysis bullosa wounds: 24-month efficacy and safety data from the EASE study. Br J Dermatol. 2023;188(Suppl 4):ljad113-144.
16.
17.
go back to reference Bageta ML, Yerlett N, McGrath JA, et al. The natural history of severe recessive dystrophic epidermolysis bullosa—4 phases which may help determine different therapeutic approaches. J Dermatol Venereol. 2021;2:20–1. Bageta ML, Yerlett N, McGrath JA, et al. The natural history of severe recessive dystrophic epidermolysis bullosa—4 phases which may help determine different therapeutic approaches. J Dermatol Venereol. 2021;2:20–1.
18.
go back to reference Denyer JE. Wound management for children with epidermolysis bullosa. Dermatol Clin. 2010;28(2):257–64.CrossRefPubMed Denyer JE. Wound management for children with epidermolysis bullosa. Dermatol Clin. 2010;28(2):257–64.CrossRefPubMed
19.
go back to reference Prodinger C, Diem A, Ude-Schoder K, et al. Profiling trial burden and patients’ attitudes to improve clinical research in epidermolysis bullosa. Orphanet J Rare Dis. 2020;15:182.CrossRefPubMedPubMedCentral Prodinger C, Diem A, Ude-Schoder K, et al. Profiling trial burden and patients’ attitudes to improve clinical research in epidermolysis bullosa. Orphanet J Rare Dis. 2020;15:182.CrossRefPubMedPubMedCentral
20.
go back to reference Hon KL, Chu S, Leung AK. Epidermolysis bullosa: pediatric perspectives. Curr Pediatr Rev. 2022;18(3):182–90.CrossRefPubMed Hon KL, Chu S, Leung AK. Epidermolysis bullosa: pediatric perspectives. Curr Pediatr Rev. 2022;18(3):182–90.CrossRefPubMed
21.
go back to reference Villar Hernández AR, Molero Alonso F, Aguado Marín ÁJ, Posada-de-la-Paz M. Transcultural validation of a Spanish version of the quality of life in epidermolysis bullosa questionnaire. Int J Environ Res Public Health. 2022;19(12):7059.CrossRefPubMedPubMedCentral Villar Hernández AR, Molero Alonso F, Aguado Marín ÁJ, Posada-de-la-Paz M. Transcultural validation of a Spanish version of the quality of life in epidermolysis bullosa questionnaire. Int J Environ Res Public Health. 2022;19(12):7059.CrossRefPubMedPubMedCentral
Metadata
Title
Oleogel-S10 in Dystrophic Epidermolysis Bullosa: A Case Series Evaluating the Impact on Wound Burden Over Two Years
Authors
Mauricio Torres Pradilla
Erick Álvarez
Mónica Novoa
Ivonne Lozano
Maribel Trujillo
Publication date
03-01-2024
Publisher
Springer Healthcare
Published in
Advances in Therapy / Issue 2/2024
Print ISSN: 0741-238X
Electronic ISSN: 1865-8652
DOI
https://doi.org/10.1007/s12325-023-02749-x

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