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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[4] Pediatric Dermatology: Ectodermal Dysplasia, I, pp. 361-363, (1995)
[5] Stevenson A.C., Kerr C.B., On the distribution of frequencies of mutation to genes determining harmful traits in man, Mulat Res, 4, pp. 339-52, (1967)
[6] Daniel E., McCurdy E.A., Shashi V., McGuist Jr. W.F., Ectoderma Dysplasia-Otolaryngologic manifestations and management, Laryngoscope, 122, 6, pp. 962-7, (2002)

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