OBJECTIVES To determine the range and prevalence of otologic disorders in patients with ectodermal dysplasia (ED) and provide a general review of its multiple otolaryngological manifestations. DESIGN Case series. SETTING Ectodermal dysplasia family conference. PATIENTS Sixty-nine individuals with ED were evaluated. The average age was 11 years (range, 1-56 years). Most were male patients ...

© 2017 Indian Dermatology Online Journal | Published by Wolters Kluwer Medknow Ectodermal dysplasias are a heterogeneous group of disorders resulting from abnormalities of structures derived from embryonic ectoderm (skin, teeth, and appendage). Rapp Hodgkin Syndrome (RHS) is a type of anhidrotic ectodermal dysplasia, which was described by Rapp and Hodgkin in 1968.[1] This syndrome is character...

The ectodermal dysplasias represent a group of inherited disorders characterized by defects in tissues that are derived from ectoderm. Freire-Maia and Pinheiro describe more than 100 different taxonomic groupings of ectodermal dysplasia across a wide spectrum of clinical presentation. The classic form of ectodermal dysplasia (Christ-Siemens-Touraine syndrome) is thought to be X-linked and invol...

chondroectodermal dysplasia (ellis-van creveld syndrome) is a rare autosomal recessive congenital abnormality. this syndrome is characterized by a spectrum of clinical findings, among which chondrodystrophy, polydactyly, ectodermal dysplasia, and congenital cardiac anomalies are the most common. it is imperative to not overlook the cardiac complications in patients with this syndrome during den...

Two brothers are documented with an ectodermal dysplasia primarily involving the teeth and hair. Both have developed cerebellar ataxia in the early teens.

Ellis-Van Creveld syndrome or chondroectodermal dysplasia is a rare autosomal recessive disorder presenting several skeletal manifestations and congenital heart malformations. Ellis-Van Creveld syndrome comprises of a tetrad of clinical manifestations of chondrodysplasia, polydactyly, ectodermal dysplasia, and cardiac defects. Here, we are presenting a very rare case of Ellis-Van Creveld syndro...