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Peer-reviewedCC BYClinical and experimental dental research

Depicting External Crown Resorption in Syndromic Amelogenesis Imperfecta Associated With Junctional Epidermolysis Bullosa.

  • Susanne Krämer1,2
  • Sebastián Véliz2,3
  • Anna-Lena Hillebrecht4
  • Ana Ortega-Pinto2
  • Colomba Besa2
  • Ignacia Fuentes5,6,7
  • Reinhard Schilke8
  • Jens P Bankstahl9
  • Elias Walter10
  • Wiebke Semper-Hogg11
  • Cristina Has1
11 affiliations
  1. 1Department of Dermatology, Medical Faculty and Medical Centre, University of Freiburg, Freiburg, Germany.
  2. 2Faculty of Dentistry, University of Chile, Santiago, Chile.
  3. 3Department of Periodontology, Dental Clinic, Sigmund Freud University, Vienna, Austria.
  4. 4Department of Prosthetic Dentistry, Center for Dental Medicine, Faculty of Medicine, Medical Center, University of Freiburg, Freiburg, Germany.
  5. 5DEBRA Chile, Santiago, Chile.
  6. 6Centro de Genética y Genómica, Facultad de Medicina Clínica Alemana, Universidad del Desarrollo, Santiago, Chile.
  7. 7Facultad de Ciencias Biológicas, Pontificia Universidad Católica de Chile, Santiago, Chile.
  8. 8Department of Conservative Dentistry, Periodontology and Preventive Dentistry, Hannover Medical School, Hannover, Germany.
  9. 9Department of Nuclear Medicine, Hannover Medical School, Hannover, Germany.
  10. 10Department of Conservative Dentistry, Periodontology and Digital Dentistry, LMU Hospital, LMU, Munich, Germany.
  11. 11Department of Oral and Maxillofacial Surgery, Center for Dental Medicine, Faculty of Medicine, Medical Center, University of Freiburg, Freiburg, Germany.
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Abstract

To characterize External Crown Resorption (ECR) in patients with Syndromic Amelogenesis Imperfecta (SAI) associated with junctional epidermolysis bullosa (JEB). A multicentre retrospective study was conducted at two centers (Germany and Chile). Analyzed variables included dentition, tooth, surface, age, severity, progression, treatment strategy, and clinical outcome. One hundred and seventy teeth affected with ECR (60 primary, 110 permanent) from 30 patients were included. Patients presented an average of 5.7 ± 4.0 affected teeth. Within this cohort, in primary dentition, incisors represented 88% of affected teeth, 8% canines, and 3% molars. In permanent dentition, 62% were incisors, 19% molars, 10% canines, and 9% premolars. Eight teeth showed two independent initiation sites, giving 178 affected surfaces in 170 teeth; 85.4% of these surfaces were incisal/occlusal, 12.9% proximal, and 1.7% vestibular/palatal. Age of detection varied from 8 months to 8.7 years (primary dentition) and from 5.2 to 22 years in permanent dentition. Severity analysis revealed that posterior teeth presented a higher proportion of severely compromised molars (61.9%), while anterior teeth presented a higher proportion of more moderate (57.4% permanent) and mild (37.7% primary) compromise. Observed progression intervals, based on 39 teeth, ranged from 4 months to 9.3 years. Imaging studies, treatment strategies, and their outcomes are presented. Restoration was associated with a lower frequency of further structural loss than preventive strategies alone. A provisional radiographic screening protocol is proposed. ECR can affect primary and permanent dentition, leading to tooth loss if left unrestored. The radiographic screening protocol for early diagnosis and treatment of ECR proposed in this article can aid in preserving teeth.

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Cite

BibTeXRIS
BibTeX
@article{kramer2026depicting,
  title = {{Depicting External Crown Resorption in Syndromic Amelogenesis Imperfecta Associated With Junctional Epidermolysis Bullosa.}},
  author = {Susanne Krämer and Sebastián Véliz and Anna-Lena Hillebrecht and Ana Ortega-Pinto and Colomba Besa and Ignacia Fuentes and Reinhard Schilke and Jens P Bankstahl and Elias Walter and Wiebke Semper-Hogg and Cristina Has},
  journal = {Clinical and experimental dental research},
  year = {2026},
  month = oct,
  doi = {10.1002/cre2.70500},
  url = {https://europepmc.org/article/MED/42956167},
  note = {CC BY},
}
RIS
TY  - JOUR
TI  - Depicting External Crown Resorption in Syndromic Amelogenesis Imperfecta Associated With Junctional Epidermolysis Bullosa.
AU  - Susanne Krämer
AU  - Sebastián Véliz
AU  - Anna-Lena Hillebrecht
AU  - Ana Ortega-Pinto
AU  - Colomba Besa
AU  - Ignacia Fuentes
AU  - Reinhard Schilke
AU  - Jens P Bankstahl
AU  - Elias Walter
AU  - Wiebke Semper-Hogg
AU  - Cristina Has
PY  - 2026
DA  - 2026/10/01
JO  - Clinical and experimental dental research
DO  - 10.1002/cre2.70500
UR  - https://europepmc.org/article/MED/42956167
AB  - To characterize External Crown Resorption (ECR) in patients with Syndromic Amelogenesis Imperfecta (SAI) associated with junctional epidermolysis bullosa (JEB). A multicentre retrospective study was conducted at two centers (Germany and Chile). Analyzed variables included dentition, tooth, surface, age, severity, progression, treatment strategy, and clinical outcome. One hundred and seventy teeth affected with ECR (60 primary, 110 permanent) from 30 patients were included. Patients presented an average of 5.7 ± 4.0 affected teeth. Within this cohort, in primary dentition, incisors represented 88% of affected teeth, 8% canines, and 3% molars. In permanent dentition, 62% were incisors, 19% molars, 10% canines, and 9% premolars. Eight teeth showed two independent initiation sites, giving 178 affected surfaces in 170 teeth; 85.4% of these surfaces were incisal/occlusal, 12.9% proximal, and 1.7% vestibular/palatal. Age of detection varied from 8 months to 8.7 years (primary dentition) and from 5.2 to 22 years in permanent dentition. Severity analysis revealed that posterior teeth presented a higher proportion of severely compromised molars (61.9%), while anterior teeth presented a higher proportion of more moderate (57.4% permanent) and mild (37.7% primary) compromise. Observed progression intervals, based on 39 teeth, ranged from 4 months to 9.3 years. Imaging studies, treatment strategies, and their outcomes are presented. Restoration was associated with a lower frequency of further structural loss than preventive strategies alone. A provisional radiographic screening protocol is proposed. ECR can affect primary and permanent dentition, leading to tooth loss if left unrestored. The radiographic screening protocol for early diagnosis and treatment of ECR proposed in this article can aid in preserving teeth.
SN  - 2057-4347
ER  -