Murakami Christiana, de Oliveira Lira Ortega Adriana, Guimarães Antônio Sérgio, Gonçalves-Bittar Daniela, Bönecker Marcelo, Ciamponi Ana Lídia
Department of Orthodontics and Pediatric Dentistry, Faculdade de Odontologia, Universidade de São Paulo, São Paulo, Brazil.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011 Aug;112(2):e11-8. doi: 10.1016/j.tripleo.2011.03.012.
Focal dermal hypoplasia (FDH), also known as Goltz-Gorlin syndrome, is an autosomal dominant disease affecting tissues derived from the ectoderm and mesoderm. Knowledge and early diagnosis of the craniofacial alterations commonly found in patients with FDH provide oral health care professionals with effective preventive and therapeutic tools. This article aims to review the craniofacial characteristics present in FDH and the main systemic manifestations that have implications for dental management, while presenting a new case of the syndrome with novel oral findings.
局灶性真皮发育不全(FDH),也称为戈尔茨-戈林综合征,是一种常染色体显性疾病,会影响源自外胚层和中胚层的组织。了解FDH患者常见的颅面改变并进行早期诊断,可为口腔保健专业人员提供有效的预防和治疗工具。本文旨在综述FDH患者的颅面特征以及对牙科治疗有影响的主要全身表现,同时报告1例具有新口腔表现的该综合征病例。