Oral Management of Recessive Dystrophic Epidermolysis Bullosa in Childhood: From Clinical Fragility to Functional Oral Care

Authors

  • Waleska Tychanowicz Kolodziejwski Multiprofessional Residency Program in Oncology and Hematology, Complexo Hospital de Clínicas, Federal University of Paraná, Curitiba-PR, Brazil; Post Graduate Program in Dentistry, Department of Stomatology, Federal University of Paraná, Curitiba-PR, Brazil https://orcid.org/0000-0001-6301-8767
  • Camila Adriane Leffa Rosa Multiprofessional Residency Program in Oncology and Hematology, Complexo Hospital de Clínicas, Federal University of Paraná, Curitiba-PR, Brazil https://orcid.org/0000-0001-6379-0976
  • Juliana Lucena Schussel Multiprofessional Residency Program in Oncology and Hematology, Complexo Hospital de Clínicas, Federal University of Paraná, Curitiba-PR, Brazil; Post Graduate Program in Dentistry, Department of Stomatology, Federal University of Paraná, Curitiba, Brazil https://orcid.org/0000-0001-5204-0782
  • Heliton Gustavo de Lima Multiprofessional Residency Program in Oncology and Hematology, Complexo Hospital de Clínicas, Federal University of Paraná, Curitiba-PR, Brazil; Post Graduate Program in Dentistry, Department of Stomatology, Federal University of Paraná, Curitiba, Brazil https://orcid.org/0000-0002-2384-5554

DOI:

https://doi.org/10.21270/archi.v14i7.6629

Keywords:

Epidermolysis Bullosa Dystrophic, Dental Care for Children, Preventive Dentistry, Low-Level Light Therapy, Dental Staff, Hospital

Abstract

Introduction: Hereditary epidermolysis bullosa (EB) is a group of heterogeneous genetic disorders characterized by skin fragility, leading to blistering, erosions, and wounds on the skin and mucous membranes in response to minimal mechanical trauma with rupture at the dermoepidermal junction. Objective: To describe the oral manifestation and dental management of a patient with recessive dystrophic EB. Case report: A 6-year-old boy, diagnosed at birth with recessive EB, was referred to the dental service of the referral hospital for follow-up. Extraoral examination revealed synechiae and hand contractures, as well as blisters and crusted lesions on the upper and lower limbs. Intraoral examination showed ulcerated and erosive lesions on the lips, blisters and ulcerations on the buccal mucosa and lateral border of the tongue, tongue depapillation, petechiae on the palate, ankyloglossia, microstomia and mixed dentition with active carious lesions, gingival erythema and biofilm accumulation. Dental management included prophylaxis sessions, restorative procedures, photobiomodulation and oral hygiene instruction. Final considerations: Oral health management in EB patients requires early integration of dental care within a multidisciplinary team, adaptation of clinical techniques, use of assistive technologies, and strong emphasis on caregiver education, in order to significantly improve the quality of life for individuals living with EB.

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References

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Published

2025-07-30

How to Cite

Kolodziejwski, W. T., Leffa Rosa , C. A., Schussel, J. L., & de Lima , H. G. (2025). Oral Management of Recessive Dystrophic Epidermolysis Bullosa in Childhood: From Clinical Fragility to Functional Oral Care. ARCHIVES OF HEALTH INVESTIGATION, 14(7), 2013–2017. https://doi.org/10.21270/archi.v14i7.6629

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Original Articles