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

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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Publication Details

Journal
ARCHIVES OF HEALTH INVESTIGATION
Published
2025-07-30
DOI
https://doi.org/10.21270/archi.v14i7.6629
Primary Topic
Skin and Cellular Biology Research
Type
article
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article

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

Juliana Lucena Schussel, Camila Adriane Leffa Rosa, Waleska Tychanowicz Kolodziejwski, Heliton Gustavo de Lima
ARCHIVES OF HEALTH INVESTIGATION
Skin and Cellular Biology Research
article

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

Juliana Lucena Schussel, Camila Adriane Leffa Rosa, Waleska Tychanowicz Kolodziejwski, Heliton Gustavo de Lima
article en

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.

ARCHIVES OF HEALTH INVESTIGATIONVol. 14(7)
Hospital de Clínicas Universidade Federal do Paraná (BR), Universidade Federal do Paraná (BR)
Openalex Percentile: Top 76%
Skin and Cellular Biology Research
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Oral Management of Recessive Dystrophic Epidermolysis Bullosa in Childhood: From Clinical Fragility to Functional Oral Care — Juliana Lucena Schussel, Camila Adriane Leffa Rosa, et al. · ARCHIVES OF HEALTH INVESTIGATION (2025) | TGRS Research Map | TGRS