RESUMO
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.
Assuntos
Displasia Ectodérmica Anidrótica Tipo 3/complicações , Displasia Ectodérmica Anidrótica Tipo 3/patologia , Anormalidades Dentárias/etiologia , Anodontia/etiologia , Anodontia/reabilitação , Criança , Fissura Palatina/etiologia , Consanguinidade , Restauração Dentária Permanente , Prótese Parcial Removível , Feminino , Humanos , Linhagem , Tratamento do Canal Radicular , Síndrome , Anormalidades Dentárias/reabilitaçãoRESUMO
The ectodermal dysplasia, ectrodactyly, cleft lip/palate syndrome (EEC syndrome) is an autosomal dominant dysplasia syndrome, whose pleiotropic effects involve mainly ectodermal structures. The most common clinical manifestations are ectodermal dysplasia, ectrodactyly , cleft lip/palate, and tear-duct anomalies. Very rarely the ectrodactyly may be absent, and skeletal abnormalities may be subtle. We present a 44-month-old girl who had features of EEC syndrome but without the classic ectrodactyly.
