Abstract
Epidermolysis bullosa hereditaria, which is characterized by a marked development of the vesicles in the ectodermal tissues, is a very rare disease. As to the oral findings, morphological abnormality of the teeth, enamel hypoplasia and abnormal oral & labial mucosa in relation to vesicles have been described.
A 2 year and 7 month old girl, who suffered from the epidermolysis bullosa hereditaria dystrophica, was reffered to the Dental Hospital of Asahi University School of Dentistry for her dental care.
The oral findings of the case were as follows.
1. Numerous congenitally missing 15 teeth, which involving both primary and permanent dentition.
2. Rough surface and hypoplasia of the enamel layer of the primary teeth was observed both clinically and histologically as well
.3. Delayed eruption in 4 primary teeth.
4. The mesio-distal width of the crowns was smaller than normal, and bilateral asymmetry of frontal teeth.
5. Orofacial asymmetry and recessive growth of both jaws were obvious.6. Apparent physical undergrowth.
