RESUMEN
Ectodermal dysplasia is a rare hereditary disorder. Its Hypohidrotic (HED) variant is also known as Chirst- Siemens-Touraine syndrome. It is inherited as an X- linked trait. Such Patients are characterized by the clinical manifestations of Hypodontia, Hypotrichosis, Hypohidrosis and a highly characteristic facial physiognomy. This article, reports a typical case of Hypohidrotic Ectodermal Dysplasia (HED) and management.
RESUMEN
Lobster-Claw syndrome is a rare autosomal dominant, hand-foot malformation with Oro-dental features. This is a rare condition and from the available reports so far, cases being reported are less than 1%. Most reports have focused on the hand-foot deformity of this syndrome. This paper highlights the typical Oro-dental features associated with this syndrome such as retained deciduous teeth, hypodontia and variation in crown size, arch length and arch width.