Hypohidrotic (Anhidrotic) ectodermal dysplasia - a rare cause of childhood Atrophic Rhinitis

被引：1

作者：

Dasgupta K.S. ^{[1
]}

Joshi S.V. ^{[1
]}

Gawarle S.H. ^{[1
]}

Murkey N.S.N. ^{[1
]}

Avad A. ^{[1
]}

机构：

[1] Department of Otorhinolaryngology, Shri Vasantrao Naik Government Medical College, Yavatmal

来源：

Indian Journal of Otolaryngology and Head and Neck Surgery | 2006年 / 58卷 / 2期

关键词：

Atrophic rhinitis; Hypohidrotic ectodermal dysplasia;

D O I：

10.1007/BF03050792

中图分类号：

学科分类号：

摘要：

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 involved1. 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. © Association of Otolaryngologists of India 2006.

引用

页码：202 / 204

页数：2

共 6 条

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