Severe Iron Deficiency Anemia in Children with Dystrophic Epidermolysis Bullosa: Three Cases.
https://doi.org/10.15690/vsp.v24i4.2933
Abstract
Background. Iron deficiency anemia is one of the most common complications of dystrophic epidermolysis bullosa (DEB). Anemia management in such cases is complicated due to many pathological factors and conditions aggravating disease course and iron absorption leading to development of anemia vicious pathological circle. Case description. Cases of anemia in children with DEB have been described. Diagnostic algorithms and therapeutic criteria have been presented. Iron (III) hydroxide sucrose was administrated, transfusion of leukoreduced red blood cell suspension was performed. Increase in hemoglobin concentration, hemodynamics stabilization, and wound healing were noted on therapy. No side effects were reported during treatment. Conclusion. Children with DEB and comorbid severe anemia require complex therapy including iron medications and transfusions. Moreover, it is crucial to update management protocols for such patients.
Supporting agencies: Not specified.
About the Authors
Nikolay N. Murashkin
National Medical Research Center of Children’s Health; Sechenov First Moscow State Medical University; Central State Medical Academy of Department of Presidential Affairs
Russian Federation
Disclosure of interest:
Russian Federation
Moscow
Disclosure of interest:
Not declared.
Ilgiz I. Yarullin
National Medical Research Center of Children’s Health
Russian Federation
Disclosure of interest:
Russian Federation
Moscow
Disclosure of interest:
Not declared.
Roman V. Epishev
National Medical Research Center of Children’s Health
Russian Federation
Disclosure of interest:
Russian Federation
Moscow
Disclosure of interest:
Not declared.
References
1. Vrozhdennyi bulleznyi epidermoliz: Clinical guidelines. Russian Society of Dermatovenerologists and Cosmetologists; Union of Pediatricians of Russia. 2020. 64 p. (In Russ).
2. 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(4):614–627. doi: https://doi.org/10.1111/bjd.18921
3. Murashkin NN, Epishev RV, Orlova OS, et al. Congenital Epidermolysis Bullosa Epidemiology among Children of Russian Federation. Voprosy sovremennoi pediatrii — Current Pediatrics. 2024;23(5):316–328. (In Russ). doi: https://doi.org/10.15690/vsp.v23i5.2808
4. Has C, Nystrom A, Saeidian AH, et al. Epidermolysis bullosa: Molecular pathology of connective tissue components in the cutaneous basement membrane zone. Matrix Biol. 2018;71–72: 313–329. doi: https://doi.org/10.1016/j.matbio.2018.04.001
5. Uitto J, Bruckner-Tuderman L, McGrath JA, et al. EB2017- progress in epidermolysis bullosa research toward treatment and cure. J Invest Dermatol. 2018;138(5):1010–1016. doi: https://doi.org/10.1016/j.jid.2017.12.016
6. Epidermolysis Bullosa: A Clinician’s Handbook. Murrell DF, ed. Springer; 2022. doi: https://doi.org/10.1007/978-3-031-05622-1
7. Zhelezodefitsitnaya anemiya: Clinical guidelines. National Hematological Society; National Society of Pediatric Hematologists and Oncologists; Russian Society of Obstetricians and Gynecologists; Russian Scientific Medical Society of Therapists; Federation of Laboratory Medicine; Russian Association of Gerontologists and Geriatricians; Endoscopic Society “REnDO”; Russian Society for the Prevention of Non-Communicable Diseases. 2024. 56 p. (In Russ).
8. Prodinger C, Reichelt J, Bauer JW, et al. Epidermolysis bullosa: Advances in research and treatment. Exp Dermatol. 2019;28(10):1176–1189. doi: https://doi.org/10.1111/exd.13979
9. Nairz M, Weiss G. Iron deficiency and anemia of chronic disease in protein malnutrition. Front Nutr. 2022;9:829425. doi: https://doi.org/10.3389/fnut.2022.829425
10. Camaschella C, Weiss G. Iron deficiency and other hypoproliferative anemias. In: Harrison’s Principles of Internal Medicine. Jameson JL, Fauci AS, Kasper DL, et al., eds. 21st ed. McGraw-Hill Education; 2022. pp. 837–844.
11. Weiss G, Ganz T, Goodnough LT. Anemia of inflammation. Blood. 2019; 133(1):40–50. doi: https://doi.org/10.1182/blood-2018-06-856500
12. Weiss G, Goodnough LT. Anemia of chronic disease. N Engl J Med. 2022;386(12):1146–1159. doi: https://doi.org/10.1056/NEJMra2115298
13. Talarico R, Cannizzo S, Lorenzoni V, et al. RarERN Path: a methodology towards the optimisation of patients’ care pathways in rare and complex diseases developed within the European Reference Networks. Orphanet J Rare Dis. 2020;15(1):347. doi: https://doi.org/10.1186/s13023-020-01631-1
14. Pope E, Lara-Corrales I, Mellerio JE, et al. Epidermolysis bullosa and chronic wounds: a model for wound bed preparation of fragile skin. Adv Skin Wound Care. 2013;26(4):177–188; quiz 189–190. doi: https://doi.org/10.1097/01.ASW.0000428864.72412.b7
15. Camaschella C. Iron deficiency. Blood. 2019;133(1):30–39. doi: https://doi.org/10.1182/blood-2018-05-815944
16. Snook J, Bhala N, Beales ILP, et al. British Society of Gastroenterology guidelines for the management of iron deficiency anaemia in adults. Gut. 2021;70(11):2030–2051. doi: https://doi.org/10.1136/gutjnl-2021-325210
17. Cappellini MD, Musallam KM, Taher AT. Iron deficiency anemia revisited. J Intern Med. 2020;287(2):153–170. doi: https://doi.org/10.1111/joim.13004
18. Shuoyan Ning MPZ. Management of iron deficiency. Hematology Am Soc Hematol Educ Program. 2019;2019(1):315–322. doi: https://doi.org/10.1182/hematology.2019000034
19. Singer CE, Vasile CM, Popescu M, et al. Role of Iron Deficiency in Heart Failure — Clinical and Treatment Approach: An Overview. Diagnostics (Basel). 2023;13(2):304. doi: https://doi.org/10.3390/diagnostics13020304
20. Simpson B, Tarango C, Lucky AW. Clinical algorithm to manage anemia in epidermolysis bullosa. Pediatr Dermatol. 2018;35(5):e319–e320. doi: https://doi.org/10.1111/pde.13587
21. Приказ Минздрава России от 25 ноября 2002 г. № 363 «Об утверждении Инструкции по применению компонентов крови» (зарегистрирован в Минюсте РФ 20.12.2002 № 4062). [Order of the Ministry of Health of Russia dated November 25, 2002 No. 363 “Ob utverzhdenii Instruktsii po primeneniyu komponentov krovi” (registered in the Ministry of Justice of the Russian Federation on December 20, 2002 No. 4062) (In Russ).]
22. Gollu G, Ergun E, Ates U, et al. Balloon dilatation in esophageal strictures in epidermolysis bullosa and the role of anesthesia. Dis Esophagus. 2017;30(3):1–6. doi: https://doi.org/10.1111/dote.12503
23. Wolf M, Rubin J, Achebe M, et al. Effects of Iron Isomaltoside vs Ferric Carboxymaltose on Hypophosphatemia in Iron-Deficiency Anemia: Two Randomized Clinical Trials. JAMA. 2020;323(5): 432–443. doi: https://doi.org/10.1001/jama.2019.22450.
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For citations:
Murashkin N.N., Yarullin I.I., Epishev R.V. Severe Iron Deficiency Anemia in Children with Dystrophic Epidermolysis Bullosa: Three Cases. Current Pediatrics. 2025;24(4):291-298. (In Russ.) https://doi.org/10.15690/vsp.v24i4.2933
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