title: Ectodermal dysplasia with alopecia, onychodysplasia, hypohidrosis, keratoderma, abnormal teeth and deafness.
abstract: The ectodermal dysplasias are a heterogeneous group of disorders with primary defect in hair, teeth, nail and sweat gland function. Numerous types have been described and several classifications exist. Here, we present a patient with ectodermal dysplasia with alopecia, dysplastic nails, hypohidrosis, sensorineural deafness, palmoplantar keratoderma, abnormal teeth and dry skin. To our knowledge, combination of all these features in ectodermal dysplasia has not been reported in the past. The etiology is unknown, but consanguinity of parents points to an autosomal recessive inheritance.
authors.affiliation: Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
authors.forename: Maryam
authors.initials: M
authors.lastname: Akhyani
authors.forename: Katrin
authors.initials: K
authors.lastname: Kiavash
mesh_terms: D000328:Adult; D000505:Alopecia / Q000150:complications; D003638:Deafness / Q000150:complications; D004476:Ectodermal Dysplasia / Q000150:complications*; D005260:Female; D006801:Humans; D007007:Hypohidrosis / Q000150:complications; D007642:Keratosis / Q000150:complications; D009264:Nails, Malformed / Q000150:complications; D014071:Tooth Abnormalities / Q000150:complications