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:: Volume 3, Issue 3 (12-2025) ::
Health Res Develop 2025, 3(3): 1-9 Back to browse issues page
Epidermolysis bullosa in children with treatment and care approach: a systematic review.
Jalal Nourmohammadi1 , Mojtaba Lotfi2 , Mahnaz Ghaljeh1 , Fereshteh Ghaljaei *3 , Mehrnaz Nazari Rad4
1- Zahedan University of Medical Sciences, Zahedan University of Medical Sciences
2- Mashhad University of Medical Sciences, Mashhad University of Medical Sciences
3- Zahedan University of Medical Sciences, Zahedan University of Medical Sciences , Ghaljaei.f51@gmail.com
4- Mashhad University of Medical Sciences, Faculty of Medicine, Mashhad University of Medical Sciences
Abstract:   (73 Views)
Background and Objective: Epidermolysis bullosa (EB) is a group of rare genetic disorders classified under genodermatoses. In children with EB, the prognosis is often poor due to complications such as renal failure, infections, and fluid and electrolyte imbalances. The association of EB with congenital anomalies has become an increasingly important concern in recent decades. The aim of this study was to systematically review existing evidence on the treatment and care strategies for children living with EB.
Materials and Methods: This study was conducted as a targeted systematic review focusing on the management and care of children with EB. A comprehensive literature search was carried out using the databases PubMed, Scopus, Web of Science, Google Scholar, and SID, employing the keywords “Epidermolysis Bullosa,” “Children,” “Treatment,” and “Care.” Studies were included if they addressed clinical management and care approaches in pediatric EB patients. After screening and removing irrelevant or duplicate articles, a total of seven studies met the eligibility criteria and were included in the final analysis.
Results: Epidermolysis bullosa imposes significant physical, emotional, and financial burdens on patients and their families. The limitations associated with this condition require both children and their caregivers to make complex decisions regarding education and daily activities. The reviewed studies consistently highlighted the negative impact of EB on quality of life, along with the considerable financial strain of ongoing care. Interventions related to wound care, pain management, and nutritional support were among the most commonly reported strategies to improve patient outcomes.
Conclusion: Epidermolysis bullosa is a rare and debilitating genetic disorder that significantly affects the lives of affected children. Although there is currently no definitive cure, interventions such as wound care, pain management, and nutritional support have played an important role in improving patients' quality of life. Ongoing research, particularly in the field of gene therapy, offers hope for more effective treatments in the future.
Keywords: epidermolysis bullosa, children, treatment, care
Full-Text [PDF 1034 kb]   (29 Downloads)    
Type of Study: Original | Subject: Special
Received: 2025/03/21 | Accepted: 2025/07/16 | Published: 2025/07/21
References
1. Delgado KZ, Navarrete RR. Epidermolysis Bullosa: Innovative Treatment with Stem Cells, a Systematic Review. 2024.doi: 10.56294/saludcyt20241193 [DOI:10.56294/saludcyt20241193]
2. Has C, Liu L, Bolling M, Charlesworth A, El Hachem M, Escámez M, et al. Clinical practice guidelines for laboratory diagnosis of epidermolysis bullosa. The British journal of dermatology. 2019;182(3):574.doi: 10.1111/bjd.18128 [DOI:10.1111/bjd.18128] [PMID] []
3. Bischof J, Hierl M, Koller U. Emerging gene therapeutics for epidermolysis bullosa under development. International journal of molecular sciences. 2024;25(4):2243.doi: 10.3390/ijms25042243 [DOI:10.3390/ijms25042243] [PMID] []
4. Lucky AW, Whalen J, Rowe S, Marathe KS, Gorell E. Diagnosis and care of the newborn with epidermolysis bullosa. Neoreviews. 2021;22(7):e438-e51.doi: 10.3390/ijms25042243 [DOI:10.3390/ijms25042243] [PMID] []
5. So JY, Teng J. Epidermolysis bullosa simplex. 2022. PMID: 20301543
6. Kueckelhaus M, Rothoeft T, De Rosa L, Yeni B, Ohmann T, Maier C, et al. Transgenic epidermal cultures for junctional epidermolysis bullosa-5-year outcomes. New England Journal of Medicine. 2021;385(24):2264-70.doi: 10.1056/NEJMoa2108544 [DOI:10.1056/NEJMoa2108544] [PMID]
7. Uitto J, Bruckner-Tuderman L, McGrath JA, Riedl R, Robinson C. EB2017-Progress in Epidermolysis Bullosa Research toward Treatment and Cure. Journal of Investigative Dermatology. 2018;138(5):1010-6.doi: 10.1016/j.jid.2017.12.016 [DOI:10.1016/j.jid.2017.12.016] [PMID]
8. Marinkovich MP, Tang JY. Gene Therapy for Epidermolysis Bullosa. Journal of Investigative Dermatology. 2019;139(6):1221-6. [DOI:10.1016/j.jid.2018.11.036] [PMID]
9. Salera S, Tadini G, Rossetti D, Grassi FS, Marchisio P, Agostoni C, et al. A nutrition-based approach to epidermolysis bullosa: Causes, assessments, requirements and management. Clinical Nutrition. 2020;39(2):343-52.doi: 10.1016/j.clnu.2019.02.023 [DOI:10.1016/j.clnu.2019.02.023] [PMID]
10. Natsuga K, Shinkuma S, Hsu C-K, Fujita Y, Ishiko A, Tamai K, et al. Current topics in Epidermolysis bullosa: Pathophysiology and therapeutic challenges. Journal of Dermatological Science. 2021;104(3):164-76.doi: 10.1016/j.jdermsci.2021.11.004 [DOI:10.1016/j.jdermsci.2021.11.004] [PMID]
11. Morgan G, Back E, Rosa D, Irwin J, Ferguson S, Carr E. PCR240 A Cross-Sectional Study of Disease Severity and Health Related Quality of Life in Epidermolysis Bullosa. Value in Health. 2022;25(12, Supplement):S436. [DOI:10.1016/j.jval.2022.09.2173]
12. Mellerio JE, Kiritsi D, Marinkovich MP, Haro NR, Badger K, Arora M, et al. Mapping the burden of severe forms of epidermolysis bullosa - Implications for patient management. JAAD International. 2023;11:224-32.doi: 10.1016/j.jdin.2023.02.016 [DOI:10.1016/j.jdin.2023.02.016] [PMID] []
13. Park M, Sachdeva M, Mistry J, Abou Ali Waked J, Abduelmula A, Maliyar K, et al. Clinical manifestations and treatment outcomes in epidermolysis bullosa pruriginosa: A systematic review. JAAD Reviews. 2024;1:1-3. [DOI:10.1016/j.jdrv.2024.05.003]
14. Bruckner AL, Losow M, Wisk J, Patel N, Reha A, Lagast H, et al. The challenges of living with and managing epidermolysis bullosa: insights from patients and caregivers. Orphanet journal of rare diseases. 2020;15:1-14.doi: 10.1186/s13023-019-1279-y [DOI:10.1186/s13023-019-1279-y] [PMID] []
15. Petrof G, Papanikolaou M, Martinez AE, Mellerio JE, McGrath JA, Bardhan A, et al. The epidemiology of epidermolysis bullosa in England and Wales: data from the national epidermolysis bullosa database. British Journal of Dermatology. 2022;186(5):843-8.doi: 10.1111/bjd.20958 [DOI:10.1111/bjd.20958] [PMID]
16. Hou P-C, Wang H-T, Abhee S, Tu W-T, McGrath JA, Hsu C-K. Investigational treatments for epidermolysis bullosa. American Journal of Clinical Dermatology. 2021;22:801-17.doi: 10.1007/s40257-021-00626-3 [DOI:10.1007/s40257-021-00626-3] [PMID]
17. Marchili MR, Spina G, Roversi M, Mascolo C, Pentimalli E, Corbeddu M, et al. Epidermolysis Bullosa in children: the central role of the pediatrician. Orphanet journal of rare diseases. 2022;17(1):147.doi: 10.1186/s13023-021-02144-1 [DOI:10.1186/s13023-021-02144-1] [PMID] []
18. Hon KL, Chu S, Leung AK. Epidermolysis bullosa: pediatric perspectives. Current pediatric reviews. 2022;18(3):182-90.doi: 10.2174/1573396317666210525161252 [DOI:10.2174/1573396317666210525161252] [PMID]
19. Prodinger C, Reichelt J, Bauer JW, Laimer M. Epidermolysis bullosa: Advances in research and treatment. Experimental dermatology. 2019;28(10):1176-89.doi: 10.1111/exd.13979 [DOI:10.1111/exd.13979] [PMID] []
20. Bruckner-Tuderman L. Newer treatment modalities in epidermolysis bullosa. Indian Dermatology Online Journal. 2019;10(3):244-50.doi: 10.4103/idoj.IDOJ_287_18 [DOI:10.4103/idoj.IDOJ_287_18] [PMID] []
21. Has C, South A, Uitto J. Molecular therapeutics in development for epidermolysis bullosa: update 2020. Molecular Diagnosis & Therapy. 2020;24(3):299-309.doi: 10.1007/s40291-020-00466-7 [DOI:10.1007/s40291-020-00466-7] [PMID] []
22. Tešanović Perković D, Bukvić Mokos Z, Marinović B. Epidermolysis bullosa Acquisita-current and emerging treatments. Journal of clinical medicine. 2023;12(3):1139. [DOI:10.3390/jcm12031139] [PMID] []
23. Delgado KZ, Navarrete RR. Epidermolysis Bullosa: Innovative Treatment with Stem Cells, a Systematic Review. 2024.doi: 10.56294/saludcyt20241193 [DOI:10.56294/saludcyt20241193]
24. Has C, Liu L, Bolling M, Charlesworth A, El Hachem M, Escámez M, et al. Clinical practice guidelines for laboratory diagnosis of epidermolysis bullosa. The British journal of dermatology. 2019;182(3):574.doi: 10.1111/bjd.18128 [DOI:10.1111/bjd.18128] [PMID] []
25. Bischof J, Hierl M, Koller U. Emerging gene therapeutics for epidermolysis bullosa under development. International journal of molecular sciences. 2024;25(4):2243.doi: 10.3390/ijms25042243 [DOI:10.3390/ijms25042243] [PMID] []
26. Lucky AW, Whalen J, Rowe S, Marathe KS, Gorell E. Diagnosis and care of the newborn with epidermolysis bullosa. Neoreviews. 2021;22(7):e438-e51.doi: 10.3390/ijms25042243 [DOI:10.3390/ijms25042243] [PMID] []
27. So JY, Teng J. Epidermolysis bullosa simplex. 2022. PMID: 20301543
28. Kueckelhaus M, Rothoeft T, De Rosa L, Yeni B, Ohmann T, Maier C, et al. Transgenic epidermal cultures for junctional epidermolysis bullosa-5-year outcomes. New England Journal of Medicine. 2021;385(24):2264-70.doi: 10.1056/NEJMoa2108544 [DOI:10.1056/NEJMoa2108544] [PMID]
29. Uitto J, Bruckner-Tuderman L, McGrath JA, Riedl R, Robinson C. EB2017-Progress in Epidermolysis Bullosa Research toward Treatment and Cure. Journal of Investigative Dermatology. 2018;138(5):1010-6.doi: 10.1016/j.jid.2017.12.016 [DOI:10.1016/j.jid.2017.12.016] [PMID]
30. Marinkovich MP, Tang JY. Gene Therapy for Epidermolysis Bullosa. Journal of Investigative Dermatology. 2019;139(6):1221-6. [DOI:10.1016/j.jid.2018.11.036] [PMID]
31. Salera S, Tadini G, Rossetti D, Grassi FS, Marchisio P, Agostoni C, et al. A nutrition-based approach to epidermolysis bullosa: Causes, assessments, requirements and management. Clinical Nutrition. 2020;39(2):343-52.doi: 10.1016/j.clnu.2019.02.023 [DOI:10.1016/j.clnu.2019.02.023] [PMID]
32. Natsuga K, Shinkuma S, Hsu C-K, Fujita Y, Ishiko A, Tamai K, et al. Current topics in Epidermolysis bullosa: Pathophysiology and therapeutic challenges. Journal of Dermatological Science. 2021;104(3):164-76.doi: 10.1016/j.jdermsci.2021.11.004 [DOI:10.1016/j.jdermsci.2021.11.004] [PMID]
33. Morgan G, Back E, Rosa D, Irwin J, Ferguson S, Carr E. PCR240 A Cross-Sectional Study of Disease Severity and Health Related Quality of Life in Epidermolysis Bullosa. Value in Health. 2022;25(12, Supplement):S436. [DOI:10.1016/j.jval.2022.09.2173]
34. Mellerio JE, Kiritsi D, Marinkovich MP, Haro NR, Badger K, Arora M, et al. Mapping the burden of severe forms of epidermolysis bullosa - Implications for patient management. JAAD International. 2023;11:224-32.doi: 10.1016/j.jdin.2023.02.016 [DOI:10.1016/j.jdin.2023.02.016] [PMID] []
35. Park M, Sachdeva M, Mistry J, Abou Ali Waked J, Abduelmula A, Maliyar K, et al. Clinical manifestations and treatment outcomes in epidermolysis bullosa pruriginosa: A systematic review. JAAD Reviews. 2024;1:1-3. [DOI:10.1016/j.jdrv.2024.05.003]
36. Bruckner AL, Losow M, Wisk J, Patel N, Reha A, Lagast H, et al. The challenges of living with and managing epidermolysis bullosa: insights from patients and caregivers. Orphanet journal of rare diseases. 2020;15:1-14.doi: 10.1186/s13023-019-1279-y [DOI:10.1186/s13023-019-1279-y] [PMID] []
37. Petrof G, Papanikolaou M, Martinez AE, Mellerio JE, McGrath JA, Bardhan A, et al. The epidemiology of epidermolysis bullosa in England and Wales: data from the national epidermolysis bullosa database. British Journal of Dermatology. 2022;186(5):843-8.doi: 10.1111/bjd.20958 [DOI:10.1111/bjd.20958] [PMID]
38. Hou P-C, Wang H-T, Abhee S, Tu W-T, McGrath JA, Hsu C-K. Investigational treatments for epidermolysis bullosa. American Journal of Clinical Dermatology. 2021;22:801-17.doi: 10.1007/s40257-021-00626-3 [DOI:10.1007/s40257-021-00626-3] [PMID]
39. Marchili MR, Spina G, Roversi M, Mascolo C, Pentimalli E, Corbeddu M, et al. Epidermolysis Bullosa in children: the central role of the pediatrician. Orphanet journal of rare diseases. 2022;17(1):147.doi: 10.1186/s13023-021-02144-1 [DOI:10.1186/s13023-021-02144-1] [PMID] []
40. Hon KL, Chu S, Leung AK. Epidermolysis bullosa: pediatric perspectives. Current pediatric reviews. 2022;18(3):182-90.doi: 10.2174/1573396317666210525161252 [DOI:10.2174/1573396317666210525161252] [PMID]
41. Prodinger C, Reichelt J, Bauer JW, Laimer M. Epidermolysis bullosa: Advances in research and treatment. Experimental dermatology. 2019;28(10):1176-89.doi: 10.1111/exd.13979 [DOI:10.1111/exd.13979] [PMID] []
42. Bruckner-Tuderman L. Newer treatment modalities in epidermolysis bullosa. Indian Dermatology Online Journal. 2019;10(3):244-50.doi: 10.4103/idoj.IDOJ_287_18 [DOI:10.4103/idoj.IDOJ_287_18] [PMID] []
43. Has C, South A, Uitto J. Molecular therapeutics in development for epidermolysis bullosa: update 2020. Molecular Diagnosis & Therapy. 2020;24(3):299-309.doi: 10.1007/s40291-020-00466-7 [DOI:10.1007/s40291-020-00466-7] [PMID] []
44. Tešanović Perković D, Bukvić Mokos Z, Marinović B. Epidermolysis bullosa Acquisita-current and emerging treatments. Journal of clinical medicine. 2023;12(3):1139. [DOI:10.3390/jcm12031139] [PMID] []
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Nourmohammadi J, Lotfi M, Ghaljeh M, Ghaljaei F, Nazari Rad M. (2025). Epidermolysis bullosa in children with treatment and care approach: a systematic review.. Health Res Develop. 3(3), 1-9.
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