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- DOI 10.18231/j.jooo.67659.1759123213
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CrossMark
When skin and smiles shatter: An extraordinary case of epidermolysis bullosa dowling-meara with devastating oro-dental involvement
- Author Details:
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Jyoti C
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Praveenkumar Ramdurg
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Naveen Srinivas
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Lingaraj S Harihar
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Mohammed Fazil
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Harshita K Arabbi
Jyoti C
Praveenkumar Ramdurg
Naveen Srinivas
Lingaraj S Harihar
Mohammed Fazil
Harshita K Arabbi
Epidermolysis Bullosa (EB) is a spectrum of rare inherited disorders characterized by skin and mucosal fragility. Oral manifestations—particularly enamel hypoplasia, blistering, and mucosal atrophy—can be severe and functionally limiting in the more aggressive subtypes such as EB simplex, Dowling-Meara (EBS-DM).Case Summary: We report the case of a 21-year-old female diagnosed with EBS-DM, the most severe variant of EB simplex caused by mutations in KRT5 or KRT14. She presented with facial and limb scarring, dystrophic nails, generalized enamel hypoplasia, over-retained deciduous teeth, multiple impacted teeth, and grossly decayed molars. Panoramic radiography confirmed periapical pathology, enamel thinning, and root shortening. Her medical history included lifelong vesiculobullous lesions and a background of consanguineous parentage. Dental management involved atraumatic extractions, fluoride therapy, and preventive protocols under a multidisciplinary team approach.Conclusion: This case emphasizes the importance of early recognition and trauma-free dental care in EB. Individualized treatment plans, psychosocial support, and genetic counseling are critical for improving function and long-term quality of life.
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