Oral lesions in recessive dystrophic epidermolysis bullosa.

OBJECTIVE

To study the prevalence of oral lesions in 35 patients diagnosed with generalized recessive dystrophic epidermolysis bullosa (RDEBg), with a quantification of their microstomia in comparison with a control group.

MATERIAL AND

METHODS

The presence of oral mucosal lesions and interincisal maximum oral aperture (MOA) was determined, classifying microstomia according to the method of Naylor, Douglass and Mix (1984).

RESULTS

Blister lesions were identified in 92% of the patients at the time of exploration--the tongue being the most affected location. Microstomia and palatal atrophy were the most prevalent sequelae (100%), while ankyloglossia, vestibular obliteration and lingual depapillation were recorded in over 90%. In 80% of the patients interincisal MOA was <30 mm (severe microstomia), while in the remaining cases maximum aperture was in the range of 31-40 mm (moderate microstomia).

CONCLUSIONS

Blister lesions were found throughout the oral mucosa in our series of patients with RDEBg, the most frequently affected location being the tongue. These lesions in turn led to invalidating sequelae such as microstomia and ankyloglossia.