title: Focal dermal hypoplasia: a case report and literature review.
abstract: 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.
authors.affiliation: Department of Orthodontics and Pediatric Dentistry, Faculdade de Odontologia, Universidade de São Paulo, São Paulo, Brazil. chrismurakami@gmail.com
authors.forename: Christiana
authors.initials: C
authors.lastname: Murakami
authors.forename: Adriana
authors.initials: A
authors.lastname: de Oliveira Lira Ortega
authors.forename: Antônio Sérgio
authors.initials: AS
authors.lastname: Guimarães
authors.forename: Daniela
authors.initials: D
authors.lastname: Gonçalves-Bittar
authors.forename: Marcelo
authors.initials: M
authors.lastname: Bönecker
authors.forename: Ana Lídia
authors.initials: AL
authors.lastname: Ciamponi
mesh_terms: D000848:Anodontia / Q000175:diagnosis; D002675:Child, Preschool; D019465:Craniofacial Abnormalities / Q000175:diagnosis*; D003481:Cuspid / Q000002:abnormalities; D017742:Dental Care for Chronically Ill; D003744:Dental Enamel Hypoplasia / Q000175:diagnosis; D042241:Early Diagnosis; D005146:Facial Asymmetry / Q000175:diagnosis; D005260:Female; D005489:Focal Dermal Hypoplasia / Q000175:diagnosis*; D005500:Follow-Up Studies; D006801:Humans; D007180:Incisor / Q000002:abnormalities; D007742:Labial Frenum / Q000002:abnormalities; D008963:Molar / Q000002:abnormalities; D021362:Palate, Hard / Q000002:abnormalities; D014071:Tooth Abnormalities / Q000175:diagnosis*; D014079:Tooth Eruption, Ectopic / Q000175:diagnosis