Sari Aysegul, Celik Salih
Faculty of Dentistry, Department of Periodontology, Hatay Mustafa Kemal University, Hatay, Turkey.
Department of Oral and Maxillofacial Surgery, TDC Dental Clinic, Antalya, Turkey.
Case Rep Dent. 2021 Mar 8;2021:6671229. doi: 10.1155/2021/6671229. eCollection 2021.
Kindler syndrome is a rare subtype of inherited epidermolysis bullosa. A 42-year-old female patient was admitted to our clinic with a complaint of tooth mobility. Multiple hypo- and hyperpigmented macules dissipated all over her body, prominent poikilodermatous changes, xerosis of the skin, and atrophy were seen in the clinical extraoral examination. Intraoral examination showed atrophy of the buccal mucosa, limited oral opening, epidermal tissue easily separated from the connective tissue, painful ulcers of the hard palate, severe periodontitis, and keratosis of the lips. All of the teeth showed mobility. After dermatologist consultation, the diagnosis of the patient was clinically identified as "Kindler syndrome." All of her teeth were extracted due to her progressive periodontal disease and late admission to our clinic. Periodontal treatment might be effective in treating and controlling oral symptoms related to the syndrome and in improving the patient's quality of life.
金德勒综合征是遗传性大疱性表皮松解症的一种罕见亚型。一名42岁女性患者因牙齿松动前来我院就诊。临床口腔外检查发现全身散在多个色素减退和色素沉着斑,明显的皮肤异色症改变、皮肤干燥及萎缩。口腔内检查显示颊黏膜萎缩、张口受限、表皮组织易与结缔组织分离、硬腭疼痛性溃疡、重度牙周炎及唇部角化病。所有牙齿均有松动。经皮肤科会诊,该患者临床诊断为“金德勒综合征”。由于其进行性牙周病及来我院就诊较晚,所有牙齿均被拔除。牙周治疗可能对治疗和控制与该综合征相关的口腔症状及改善患者生活质量有效。