Oral Health Considerations for the Management of Patients with Junctional Epidermolysis Bullosa

Abstract

Epidermolysis bullosa (EB) is a group of hereditary disorders characterized by the mechanical fragility of skin and the appearance of blisters, bullae, and ulcers. This case report presents a 10-year-old boy with junctional EB (JEB) diagnosed at 2 months of age, with skin and oral presentation. There were scar marks of the healed lesion on the forehead, ear, neck, body, palms, and feet. There were no nails in any of the fingers of hands and feet. Ulcers were seen on the lateral border of the tongue, and caries involving pulp were found in three permanent molars. Preventive procedures were performed and root stumps were extracted. Limited mouth opening posed a problem for root canal treatment of permanent molars. Management of EB is challenging and requires a multidisciplinary team approach.

Keywords

• blisters
• bullae
• fragile skin
• junctional epidermolysis bullosa