Congenital Ectodermal Dysplasia

Question 1

Congenital Ectodermal Dysplasia

Answer 1

Skin condition representing group of rare hereditary disorders involving tissue of ectodermal origin.

Teeth are completely or partially absent and often the hard and nails are affected.

Question 2

Ectrodactyly-ectodermal dysplasia-clefting syndrome

Answer 2

congenital skin condition that is inherited as an autosomal dominant trait involving both ectodermal and mesodermal tissues, consisting of ectodermal dysplasia associated with hypo pigmentation of skin and hair, scanty hair, and eyebrows, absence of eyelashes, nail dystrophy, hypodontia and microdontia, ectrodactyly (split hand, cleft hand, lobster claw hand), and cleft lip and palate.

Appears to be caused by a defect in the P63 gene coding a transcription factor.