A Case of Anhidrotic Ectodermal Dysplasia with Atopic Dermatitis / 대한피부과학회지

ABSTRACT

Anhidrotic ectodermal dysplasia(AED) is a rare hereditary disorder characterized by hypohidrosis or anhidrosis, hypotrichosis, dental hypoplasia and characteristic facies. Additional less consistent symptoms include nail dystrophy, hyperkeratosis of the palms and soles, cleft palate, hyperplasia of facial sebaceous glands, susceptibility to atopic dermatitis, dryness of mouth and eyes, and hypoplasia of mucous and mammary glands. In general, the inheritance of this syndrome is determined by an X-linked recessive gene, and several hundred cases, of which over 90% are male, have been reported in many differnt races. We experienced a case of AED associated with atopic dermatis.