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. 2025 Dec 5;25(1):1904.
doi: 10.1186/s12903-025-07457-2.

Oral health of patients with epidermolysis bullosa compared to healthy controls - a retrospective study from a specialized center

Tara Groß #  1 , Sophia Aurelia Stocker #  2 , Konstantin J Scholz  1   3 , Cristina Has  4 , Kirstin Vach  5 , Fabian Cieplik  1
Affiliations

Oral health of patients with epidermolysis bullosa compared to healthy controls - a retrospective study from a specialized center

Tara Groß et al. BMC Oral Health. .

Abstract

Background: Epidermolysis bullosa (EB) is a rare genetic disorder characterized by skin and mucosal fragility, leading to significant oral health challenges due to mucosal blisters, pain, up to microstomia and ankyloglossia in severe cases. This retrospective study aimed to assess oral health in EB patients compared to a healthy control group to develop tailored dental protocols.

Methods: Medical records and panoramic x-rays of EB patients (n = 40) treated at the Center for Dental Medicine, University Medical Center Freiburg (2014-2024), were analyzed and compared to a healthy control group (n = 40) with similar age- and gender distribution. Oral health was evaluated using DMFT/dmft index and treatment data including extractions and fillings. Statistical analysis was performed using the Wilcoxon-Mann-Whitney test (α = 0.05).

Results: 37 EB patients (median (25-75%) age: 10 (5;17) years) and 37 controls (median age: 9 (5;16) years) were included after three exclusions each. EB patients exhibited significantly higher DMFT/dmft scores (p < 0.0001), more carious teeth (p < 0.0001), resulting in more tooth extractions (p < 0.0001) compared to the control group.

Conclusions: EB patients had significantly higher caries burden and required more extractions compared to controls. The findings of this study highlight the necessity of specific dental interventions emphasizing preventive care.

Keywords: Dental treatment; Epidermolysis bullosa; General anesthesia; Oral health; Retrospective study; Tooth extractions.

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Conflict of interest statement

Declarations. Ethics approval and consent to participate: The study design was approved by the Ethics Committee of the Albert-Ludwigs-University of Freiburg, Germany (Number: 24-1317-S1-retro) in accordance with the 1964 Helsinki Declaration and its later amendments and comparable ethical standards. As this was a retrospective analysis of pseudonymized data, written informed consent of all patients was not necessary. Consent for publication: Written informed consent was obtained from the respective patients for the use of clinical images shown in Figs. 1, 3 and 4. Competing interests: The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Exemplary picture of a DEB patient (7 years old, female), fragile extra-oral skin, intra- and peri-oral blistering. Alterations in dental enamel, indicative of hypoplasia, are visible. There is a noticeable plaque accumulation, likely accompanied by marginal gingival inflammation. The lip skin appears reddened and bears marks of previous scarring. Even with minimal mouth opening, the lips exhibit circular tension, suggesting microstomia
Fig. 2
Fig. 2
Association between age and DMFT/dmft index for the test group (EB) and the control group according to oral hygiene status. The x-axis represents the patients’ age (in years), and the y-axis shows the DMFT/dmft score, indicating the total number of decayed (D/d), missing (M/m), and filled (F/f) teeth. Colors indicate the recorded oral hygiene categories: green = good, blue = medium, and pink = poor. For visualization the linear regression line showing the association of age and DMFT/dmft within each group was presented
Fig. 3
Fig. 3
Panoramic x-ray of a 14-year-old male JEB patient, presenting generalized severe enamel hypoplasia. Enamel alterations are also visible in non-erupted teeth 37, 38, 47, 48. Upper incisors revealed extensive restorations
Fig. 4
Fig. 4
Exemplary picture of a JEB patient (14-year-old, male) with generalized enamel hypoplasia
See this image and copyright information in PMC

References

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