Abstract
Epidermolysis bullosa is a group of rare genetic disorders characterised by skin and mucous membrane fragility and systemic manifestations of variable severity. We report a case of dystrophic epidermolysis bullosa in an 18-year-old male patient who presented to the Department of Oral Health at Sultan Qaboos University Hospital, Muscat, Oman, in 2015 with recurrent dental pain and infections. Due to the poor dental status of the patient and anticipated operative difficulties due to microstomia and limited mouth opening, the patient underwent full dental clearance under general anaesthesia. This article discusses the dental and anaesthetic challenges encountered during the management of this patient and provides a brief literature review.
Highlights
Epidermolysis bullosa is a group of rare genetic disorders characterised by skin and mucous membrane fragility and systemic manifestations of variable severity
We report a case of dystrophic epidermolysis bullosa in an 18-year-old male patient who presented to the Department of Oral Health at Sultan Qaboos University Hospital, Muscat, Oman, in 2015 with recurrent dental pain and infections
This report details the successful full dental clearance under GA of an adult with dystrophic EB (DEB) which resulted in satisfactory healing and minimal scarring
Summary
Anaesthesia (GA) at different hospitals, including balloon dilatation of oesophageal strictures, incisional release of pseudosyndactyly and insertion and removal of a percutaneous endoscopic gastrostomy tube He applied silver sulfadiazine ointment on the skin lesions and used artificial tears on a daily basis. The patient was accompanied by his parents and appeared to have normal cognitive function He was initially anxious and uncooperative during the examination due to severe dental pain. The patient had ankyloglossia and a smooth tongue with obliteration of the buccal and lingual vestibules His dental status was poor, with multiple advanced dental caries in most of his teeth; these had caused the formation of numerous dental sinuses which discharged to the oral cavity. At the time of writing, the patient was currently in the process of undergoing prosthetic dental rehabilitation
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