Sammanfattning
Epidermolysis bullosa (EB) is a group of rare hereditary and
chronically skin disorders, characterized by bullae and fragility
of the skin and mucous membranes due to friction or minor
trauma. The severity varies from mild, with hardly any functional
impairment, to the most severe forms of EB with life-threatening
complications and fatal outcome. In general, the symptoms of
the oral mucosa manifest as vesiculobullous and erosive lesions
that can vary from small discrete erosions/bullae to widespread
erosive changes. There is also a risk of developing squamous
cell carcinoma. If the patient has Junctional EB, the tooth formation
can be affected and enamel hypoplasia seen. The approach
to patients with EB and oral symptoms has today largely
focus on individually organized preventive dentistry, aimed at
minimizing the need for treatment. Dental treatment should be
carried out as gently as possible to prevent traumatization of the
mucous membranes and thereby prevent the development of
bullae, and is mainly determined by the patient’s general condition,
the patient’s cooperation in the clinic and at home, the
nutritional status of the patient, and the oral hygiene. Pediatric
dentists play a special role in early prevention and intervention
to minimize the risk of the development of caries and maintaining
oral health. Patients with severe forms of EB, and with many
oral manifestations may need a specialized dental treatment.