Dental implications of Tooth-Nail dysplasia (Witkop syndrome): a report of an affected family and an approach to dental management

G M Wicomb; L X G Stephen; P Beighton

doi:10.17796/jcpd.28.2.p273r7k4tp561285

Dental implications of Tooth-Nail dysplasia (Witkop syndrome): a report of an affected family and an approach to dental management

J Clin Pediatr Dent. 2004 Winter;28(2):107-12. doi: 10.17796/jcpd.28.2.p273r7k4tp561285.

Authors

G M Wicomb¹, L X G Stephen, P Beighton

Affiliation

¹ University of the Western Cape, Dental Unit for Genetic Disorders, Red Cross Children's Hospital, Rondebosch, Cape Town, South Africa. gwicomb@uwc.ac.za

PMID: 14969367
DOI: 10.17796/jcpd.28.2.p273r7k4tp561285

Abstract

Tooth-Nail dysplasia is a rare genetic disorder, which is classified as an ectodermal dysplasia. Diagnostic differentiation from other conditions in this category is necessary for effective dental management and genetic counseling. The oro-dental and clinical manifestations of Tooth-Nail dysplasia in an affected male infant and his father are documented. Other family members have the condition and pedigree data are in keeping with autosomal dominant inheritance. A comprehensive approach to the dental management of an affected child is proposed.

Publication types

Case Reports
Research Support, Non-U.S. Gov't

MeSH terms

Adult
Child, Preschool
Composite Resins
Dental Restoration, Permanent
Ectodermal Dysplasia / complications*
Humans
Male
Nails, Malformed / etiology*
Pedigree
Syndrome
Tooth Abnormalities / etiology*
Tooth Abnormalities / therapy*
Tooth, Deciduous / abnormalities

Substances

Composite Resins

Associated data

OMIM/189500