Affiliation:
1. İSTANBUL ÜNİVERSİTESİ, DİŞ HEKİMLİĞİ FAKÜLTESİ
2. İSTANBUL ÜNİVERSİTESİ, DİŞ HEKİMLİĞİ FAKÜLTESİ, DİŞ HEKİMLİĞİ (DR)
3. İSTANBUL ÜNİVERSİTESİ, DİŞ HEKİMLİĞİ FAKÜLTESİ, DİŞ HEKİMLİĞİ PR.
Abstract
Background: This case report aims to contribute to the understanding of oral findings and dental approaches in dystrophic epidermolysis bullosa, a rare hereditary disease.
Cases: We reported four congenital dystrophic epidermolysis bullosa cases from two separate families with clinical and radiographic findings in this case report. Erosive-cicatrized lesions were observed on the skin in all cases. Pseudosyndactyly was present in two cases. In the intraoral examination, reduction in mouth opening, obliteration of the vestibule sulcus, ankyloglossia, and many decayed teeth were observed in all cases. There was no intraoral lesion in two cases, but loss of lingual papillae. Intraoral bullae and erosive lesion formation were observed in two cases.
Conclusions: Dystrophic epidermolysis bullosa is a rare hereditary disease. Lesions are usually seen on the skin, but mucosal involvement is also common. For this reason, dentists should know the oral manifestations of this disease and the dentist's approach in detail.