title: Hypohidrotic (Anhidrotic) Ectodermal dysplasia - A rare cause of childhood Atrophic Rhinitis".
abstract: Hypohydrotic (Anhidrotic) Ectodermal dysplasia is a rare hereditary genodermatosis characterized by a triad of defects including hypohidrosis hypotrichosis and anomalous dentition. Mainly ectodermal structures are involved such as epidermis and its annexes (hair & nails), although non-ecotodermal tissue may also became involved(1). ENT involvement in such cases is a consequence of the defect involving the eccrine and sebaceous glands. A case of a 3 years old female child suffering from hypohidrotic ectodermal dysplasia with features of Atrophic Rhinitis from infancy & early childhood is reported here. Management of these patients is mainly supportive and otolaryngologist is a vital component of the health care team managing such patients.
authors.affiliation: Department of Otorhinolaryngology, Shri Vasantrao Naik Government Medical College, Yavatmal.
authors.forename: S
authors.initials: S
authors.lastname: Das Gupta
authors.forename: K S
authors.initials: KS
authors.lastname: Dasgupta
authors.forename: Samir V
authors.initials: SV
authors.lastname: Joshi
authors.forename: Kanchan
authors.initials: K
authors.lastname: Lanjewar
authors.forename: Neeraj
authors.initials: N
authors.lastname: Murkey
authors.forename: Surendra H
authors.initials: SH
authors.lastname: Gawarle
authors.forename: N S N
authors.initials: NS
authors.lastname: Murkey
authors.forename: Atul
authors.initials: A
authors.lastname: Avad
keywords: Atrophic Rhinitis; Hypohidrotic Ectodermal Dysplasia