Dental and anaesthetic management of children with dystrophic epidermolysis bullosa

Abstract

Dystrophic EB is a rare genetic disorder in which vesiculobullous lesions occur with erosions and scarring of cutaneous and extracutaneous surfaces in the oral cavity. Poor oral hygiene results from efforts to avoid mucosal trauma, resulting in decay. The comprehensive care of children with dystrophic EB impacts not only dental management but anaesthesia administration as well.

Oral Health Care for Patients with Epidermolysis Bullosa ‐ Best Clinical Practice Guidelines

“…D The use of stainless steel crowns has been reported as a successful approach in children with RDEB and JEB 4,5,22,41 .…”

“…Healing of the oral tissues occurs gradually after one to 2 weeks 16,21,41 . Healing of the alveolar sockets seems to be uneventful 6,9 .…”

“…The scarring of generalized RDEB can give rise to obliteration of the 4,7,9,11,12,18,19,22,23,27,28,31,36,41 and hence has the potential to compromise oral hygiene procedures, dental treatment, and the wearing of removable prosthetic appliances (Image 26).…”

“…Tongue papillae are absent. 4,5,7,9,18,22,28,30,36,41 (Image 24) Ankyloglossia. Ankyloglossia presumably secondary to ulceration is common, indeed may affect all patients (Image 25) 1,4,5,7,12,16,18,19,22,23,28,31,77 .…”

“…D Haemostasis can be achieved with gentle pressure using gauze packs 9,41 . These should be wet to avoid tissue adherence.…”