Open AccessHypohidrotic (Anhidrotic) Ectodermal dysplasia — A rare cause of childhood Atrophic Rhinitis”
Author(s) -
K. S. Dasgupta,
Samir Joshi,
Surendra H. Gawarle,
N. S. N. Murkey,
Atul Avad
Publication year - 2006
Publication title -
indian journal of otolaryngology and head and neck surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.229
H-Index - 22
eISSN - 2231-3796
pISSN - 0973-7707
DOI - 10.1007/bf03050792
Subject(s) - hypohidrotic ectodermal dysplasia , ectodermal dysplasia , medicine , genodermatosis , hypotrichosis , dermatology , otorhinolaryngology , dentistry , surgery , genetics , biology , gene
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.