Ectodermal dysplasia (ED) is a rare group of inherited disorders with defects in the development one or more tissues derived from primary embryonic ectodermal derivatives. The inclusion ED does not include all derivatives and manifested defect skin, hair, teeth, eccrine glands. Dry frontal bossing, scanty eyebrows, hair follicles are common features seen these patients. patient may suffer high ...

Background Ectrodactyly ectodermal dysplasia-cleft syndrome is a rare genetic syndrome with an incidence of 1/90,000 live births, characterized by cleft lip and palate, severely hypoplastic maxilla, and hypodontia. Patients diagnosed with ectrodactyly ectodermal dysplasia-cleft syndrome suffer from a severely hypoplastic maxilla that is highly difficult to treat using traditional orthognathic m...

X-linked hypohidrotic ectodermal dysplasia (XLHED) caused by variants in the EDA gene represents the most common ectodermal dysplasia in humans. We investigated three male mixed-breed dogs with an ectodermal dysplasia phenotype characterized by marked hypotrichosis and multifocal complete alopecia, almost complete absence of sweat and sebaceous glands, and altered dentition with missing and abn...

Anhidrotic Ectodermal Dysplasia In an extensive review of different forms of hereditary ectodermal dysplasia, Cockayne (I933) recognized two types of inheritance for the variety characterized by anhidrosis, hypotrichosis, and complete or partial anodontia. He postulated an X-linked recessive gene in families where males alone were affected and an autosomal dominant gene where females were invol...

We report a case of a six years age girl who presented to our out patients department with the history and findings suggestive of recurrent respiratory tract infections. She was also noticed to have: non homogeneous hyperpigmented patches on the face since three months of age, sparse hair on the scalp and eyebrows, conical peg like teeth and delayed dentition, prominent and low set ears, perpet...

Background. Female patients presenting amastia associated with ectodermal dysplasia are not frequently encountered, but they are of great clinical interest and surgically demanding. Traditionally, skin alterations related to Ectodermal Dysplasia have addressed plastic surgeons to perform a two-stage approach in amastia associated with this congenital pathologic condition. This article describes...

INTRODUCTION Ectrodactyly-ectodermal dysplasia-cleft lip or palate syndrome (OMIM No. 129900) is characterized by the triad of ectrodactyly, ectodermal dysplasia and facial clefting (of the lip and/or palate). Holoprosencephaly denotes a failure in the division of the embryonic forebrain (prosencephalon) into distinct lateral cerebral hemisphere. The association between ectrodactyly-ectodermal ...

Purpose The American Academy of Pediatric Dentistry (AAPD) recognizes that pediatric dentists are uniquely qualified to manage the oral health care needs of children with heritable dental developmental anomalies. These children have multiple, complex problems as their dental conditions affect both form and function and can have significant psychological impact. These conditions may present earl...