|
KMID : 0032220220340040291
|
|
Annals of Dermatology
2022 Volume.34 No. 4 p.291 ~ p.296
|
|
|
Focal Dermal Hypoplasia (Goltz Syndrome): A Case Report Showing a Wide Variety of Systemic and Oral Manifestations
|
|
Nobre Atila Vinicius Vitor
Taba Mario Junior
Silva Alfredo Ribeiro
de Souza Sergio Luis Scombatti
Motta Ana Carolina Fragoso
|
|
|
Abstract
|
|
|
|
Focal dermal hypoplasia (FDH), also known as Goltz syndrome, consists of an unusual genodermatosis that affects tissues of ectodermal and mesodermal origin and various organs and systems, especially skin, bones, eyes, and oral cavity. While systemic manifestations of FDH have been well documented, the oral manifestations have not been extensively discussed. We present a 22-year-old female patient with history of FDH that showed a variety of systemic and oral manifestations. FDH was diagnosed at birth based on cutaneous alterations. Extra and intraoral examination showed facial asymmetry, lip and perioral atrophy, upper lip papilloma, malocclusion, enamel hypoplasia, and gingival hyperplasia. Mucosal lesions, periodontal diseases, and malocclusion were treated by oral surgery, periodontal therapy and orthodontic treatment, respectively. Although FDH is an uncommon syndrome, health professionals should be aware of its systemic and oral manifestations to establish an early diagnosis and adequate treatment.
|
|
|
KEYWORD
|
|
|
Diagnosis, Focal dermal hypoplasia, Genetics, Oral manifestations
|
|
|
FullTexts / Linksout information
|
|
|
|
|
Listed journal information
|
|
   
|
|