Dystrophic epidermolysis bullosa: Oral findings and problems

Abstract

Dystrophic epidermolysis bullosa (DEB) is one of the three major types of epidermolysis bullosa (EB), an inherited cutaneous disease with blister formation following minor trauma. A subtype of DEB is recessive dystrophic epidermolysis bullosa, Hallopeau-Siemens type (RDEB-HS), where marked scarring leads to deformities of extremities. In RDEB-HS the mucous membranes may also be involved and form adhesions with ankyloglossia and microstomia. Oral hygiene is difficult. A 7-year-old boy with RDEB-HS was brought to the Johannes Gutenberg University dental clinic with dental pain. He had multiple carious lesions, poor oral hygiene and gingivitis. Because he was noncompliant and had microstomia, he required dental therapy under general anesthesia. The recall visits over the past two years had demonstrated that the dental health of this patient with RDEB-HS could be maintained by means of improved oral home care, using antibacterial agents.

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

“…Chlorhexidine D Chlorhexidine 0.12% has been widely advocated for oral disease prevention in patients with EB 5,7,10,11,16,19,20 . It has shown to be effective for candida while ineffective for caries control.…”

“…The following suggestions can help determine the appropriate toothbrush for each patient: (a) Small head 5,7,8,11,13 . (b) Soft bristle 5,8,11,13,18 .…”

“…Every 3-6 months may be sufficient for some patients, and for others, monthly appointments may be necessary 3,5,15,22,27 . The review sessions should be aimed at 3,7,15,19,22 : (a) Caries prevention ⁄ early diagnosis. Any unusual ulcer or white or red patches should be biopsied to ensure that these do not represent pre-cancer or cancer in the mouth.…”

“…Maintaining a functional dentition reduces the potential for oral and oesophageal soft tissue damage through more efficient mastication 7,8 ; however, a nutritional consultation is important, as advice must be given on the nutritional content of the diet and the consistency of the food. 4) Improved phonetics when anterior teeth are restored, allowing for better positioning of the tongue.…”

“…This enables dentists to start preventive programmes and reduces the risk of developing dental diseases 14 . Many case reports have shown that patients visit the dentist only when they already have several carious lesions or pain 7,11,15,16 .…”