Abstract
Epidermolysis Bullosa Hereditaria is a rare disease of the skin and/or mucous membranes. This disease is characterized by a marked development of vesicles and bullae which arise as a result of a slight trauma and may appear spontaneously. A number of classifications have been mentioned in the reports concerning this disease. The diagnosis of this case was based on the classification of Hashimoto (1981), and the patient was diagnosed as epidermolysis bullosa dystrophica Hallopeau-Siemens, which includes several oral changes and problems.
This report concerns a clinical observation of a case of epidermolysis bullosa dystrophica Hallopeau-Siemens. A boy aged 6 years was examined from the dental point of view.
The findings were as follows:
1. Physical growth was retarded, and anemia was found.
2. At the anterior fontanelle, the demineralization of the bone was seen on a radiographic examination of the skull.
3. Based on the cephalometric diagnosis, it was found that the growth in both jaws was delayed.
4. Severe dental caries had attacked all deciduous teeth.
5. The form, number, eruption and development of the permanent teeth were normal, and enamel hypoplasia was not observed.
6. Microstomia was found. The buccal and labial vestibules and palatal rugae were obliterated with scar tissue. The tongue was bound down to the oral floor, with consequent impairment of tongue mobility.
7. Local anesthesia caused no abnormality of the oral mucous membranes.
