Oral Manifestations of Dystrophic Epidermolysis Bullosa: Four Case Reports

Year 2024, Volume: 11 Issue: 2, 218 - 222, 19.08.2024

Rabia Duman Tepe , Melek Tufan Yücesoy , Hülya Çakır Karabaş

https://doi.org/10.15311/selcukdentj.1370884

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.

Keywords

ankyloglosia, dental caries, dystrophic epidermolysis bullosa, epidermolysis bullosa, microstomia

Distrofik Epidermolizis Büllozanın Oral Bulguları: Dört Vaka Sunumu

Abstract

Amaç: Bu vaka raporu, nadir görülen kalıtsal bir hastalık olan distrofik epidermolizis büllozanın ağız bulguları ve diş hekimliğine yaklaşımı hakkındaki bilgilere katkıda bulunmayı amaçlamaktadır.

Olgu Sunumu: Bu olgu sunumunda iki ayrı aileden dört konjenital distrofik epidermolizis bülloza olgusunu klinik ve radyografik bulgularıyla bildirdik. Tüm vakalarda ciltte eroziv skatrize lezyonlar gözlendi. İki olguda psödosindaktili mevcuttu. Ağız içi muayenede tüm vakalarda ağız açıklığında azalma, vestibül sulkusta obliterasyon, ankiloglossi ve çok sayıda dişte çürük gözlendi. İki olguda ağız içi lezyon yoktu ancak lingual papilla kaybı vardı. İki olguda ağız içi bül ve eroziv lezyon oluşumu gözlendi.

Sonuç: Distrofik epidermolizis bülloza nadir görülen kalıtsal bir hastalıktır. Lezyonlar genellikle deride görülmekle birlikte mukozal tutulum da sıktır. Bu nedenle diş hekimlerinin bu hastalığın ağızdaki belirtilerini ve diş hekiminin yaklaşımını detaylı olarak bilmesi gerekmektedir.

Keywords

ankyloglosia, diş çürüğü, distrofik epidermolizis bülloza, epidermolizis bülloza, mikrostomi

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