Rapp-Hodgkin syndrome: clinical and dental findings.

Abstract

Rapp-Hodgkin syndrome is a rare form of ectodermal dysplasia involving the hair, eyes, sweat glands, nails, teeth and palate. This syndrome is characterized by stiff sparse hair with the appearance of steel wool, sparse eyebrows and lashes, cleft palate, absence of lacrimal punctae, epiphora, a decreased number of sweat glands, and dystrophic nails. Cleft palate, hypodontia, abnormal tooth shape, multiple caries, delayed eruption of teeth are the main oral manifestations. In this paper we describe the clinical and dental findings of this syndrome in a 7 year old girl referred to the dental clinic because of severe tooth ache. The dental treatment included root canal treatment, composite restorations, extractions and removable prostheses. This paper highlights features of Rapp-Hodgkin Syndrome and its dental rehabilitation.

• Authors: Gul Tosun, Ulku Elbay
• Journal: The Journal of clinical pediatric dentistry (J Clin Pediatr Dent) Vol. 34 Issue 1 Pg. 71-5 ( 2009) ISSN: 1053-4628 [Print] Singapore
• PMID: 19953814 (Publication Type: Case Reports, Journal Article)

Topics

• Anodontia (etiology, rehabilitation)
• Child
• Cleft Palate (etiology)
• Consanguinity
• Dental Restoration, Permanent
• Denture, Partial, Removable
• Ectodermal Dysplasia 3, Anhidrotic (complications, pathology)
• Female
• Humans
• Pedigree
• Root Canal Therapy
• Syndrome
• Tooth Abnormalities (etiology, rehabilitation)