The Mouth

During infancy, blisters frequently occur in the mouth with all types of EB. Although the oral mucosa may remain fragile throughout life, babies with EB simplex generally experience a dramatic reduction of oral blistering with weaning from the bottle. Those with junctional and dystrophic EB often continue to blister, and scarring may result. When scarring is present in the mouth, microstomia and ankyloglossia may develop.

Microstomia is an abnormal smallness of the mouth. This is caused by scarring of the oral mucosa, particularly of the buccal surface (the inner lining of the cheeks and lips). Surgery to correct microstomia may or may not be successful, as the mucosa tends to heal and re-scar in the same or a similar manner. Daily stretching of the mouth has been reported to provide minimal help. Placing tongue blades between the front teeth and rocking them in an up-and-down motion will help stretch the mouth opening. Every effort should be made to maintain as wide an opening as possible.

Practicing routine oral hygiene is difficult because the oral mucosa is fragile. Brushing and flossing efforts often result in pain, bleeding, erosions and further scarring of the oral mucosa. Severe tooth decay often results and is difficult, if not impossible, to treat, due, in part, to the limited space in which a dentist can work. (Refer to section on dental care.) Enormous effort and commitment is required to manage this ongoing problem.

Ankyloglossia is a condition that results from the tongue scarring and fusing to the floor of the mouth. The affected individual is not able to stick out his/her tongue, and it appears that the tongue is shorter than normal. This sometimes causes problems with eating and speaking; individuals usually compensate with little difficulty, however, and have little or no problem adapting.

There are several thousand tastebuds on the tongue. Tastebuds are bundles of nerve endings that transmit messages to the brain by chemical reactions. The messages allow people to perceive different tastes, including sweet, salty, sour, bitter and umami. Tastebuds can be dulled or damaged by wounds and scar tissue. Non-functional tastebuds, along with lesions and pain in the mouth, may lead to decreased desire to eat and try new foods. Children with EB often do not eat well.

Overall, caution should be used when hard-crusted and crispy foods such as crackers, chips or pizza are eaten. It may be necessary to avoid these foods in order to prevent injury to the mouth, throat and esophagus. Very warm and hot foods also may cause blistering and should be avoided.

Oral pain presents a significant problem for people with EB. Those with severe EB have an increased caloric requirement for wound healing and a compromised ability to ingest food. When painful oral wounds are present, the nutritional challenge becomes more difficult.

These are suggestions for oral pain relief: