Inherited epidermolysis bullosa: an update and suggested dental care considerations.

BACKGROUND

Epidermolysis bullosa (EB) is a skin disease characterized by epithelial fragility that leads to blistering and erosion of the skin and mucosae. The authors conducted a literature review to provide an update on oral manifestations and dental care of patients with EB. Literature Search. The authors reviewed the dental literature on EB in relation to clinical findings and provision of dental care. They searched textbooks and three databases: MEDLINE, Cochrane Library and Embase. The authors did not impose any date or publication status restrictions. They searched all databases up to August 2010.

RESULTS

The literature review revealed that four major groups and 32 subtypes of EB can be distinguished on the basis of the ultrastructural characteristics of skin cleavage, genetic mode of transmission and clinical phenotype. Oral manifestations differ in frequency and severity according to the disease subtype, but the most common are bullae, which leave painful ulcers on rupture, followed by scarring and tissue contraction. Although good oral health status is essential to maintaining oral function, dental treatment can induce new lesions and be hindered by the sequelae of existing lesions.

CLINICAL IMPLICATIONS: Dental treatment in patients with EB requires a multidisciplinary approach. Dental procedures must be minimally traumatic, and the effectiveness of treatment is determined mainly by the patient's general health, cooperation in the dental office and at home, oral hygiene and diet.