Mestrallet Pierre, Wardani Adnane, Evrard Laurence
Department of Stomatology, Oral and Maxillofacial Surgery Hôpital Erasme Université libre de Bruxelles Brussels Belgium.
Department of Dentistry Hôpital Erasme Université libre de Bruxelles Brussels Belgium.
Clin Case Rep. 2022 May 16;10(5):e05813. doi: 10.1002/ccr3.5813. eCollection 2022 May.
Inherited epidermolysis bullosa (EB) is a disease that causes epithelium fragility due to a protein anomaly caused by a genetic mutation. Epidermolysis bullosa clinical manifestations are bullae and cutaneous-mucosal erosions. Epidermolysis bullosa is a rare disease, with different clinical presentations depending on the type and subtype. The stomatological treatment depends on the oral manifestations and EB types. There is no high level of recommendations due to the limited cases described in the literature. We describe an implant-supported dento-maxillary rehabilitation of a 49-year-old patient suffering from a newly diagnosed hereditary recessive EB with disabling oral manifestations. In the current case, the diagnosis of recessive dystrophic epidermolysis bullosa has been confirmed, and adequate dental care was carried out taking into account the disease constraints.
遗传性大疱性表皮松解症(EB)是一种由于基因突变导致蛋白质异常而引起上皮细胞脆弱的疾病。大疱性表皮松解症的临床表现为水疱和皮肤黏膜糜烂。大疱性表皮松解症是一种罕见疾病,根据类型和亚型不同有不同的临床表现。口腔治疗取决于口腔表现和EB的类型。由于文献中描述的病例有限,尚无高水平的推荐意见。我们描述了一例49岁新诊断为遗传性隐性EB且伴有严重口腔表现患者的种植体支持式牙上颌修复。在当前病例中,隐性营养不良型大疱性表皮松解症的诊断已得到证实,并在考虑疾病限制因素的情况下进行了充分的牙科护理。