Hypohidrotic ectodermal dysplasia with atrophic rhinitis and nasal myiasis |
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Authors: | J. Madana Deeke Yolmo S. Gopalakrishnan S.K. Saxena A.K. Nath V. Ilamaran |
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Affiliation: | Department of Otorhinolaryngology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry 605 006, India |
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Abstract: | Hypohidrotic/anhidrotic ectodermal dysplasia is a rare inherited disorder characterized by hypohidrosis/anhidrosis, hypotrichosis, dysodontia and heat intolerance. Most common mode of transmission is X-linked recessive, showing complete expression in males, and only partial manifestations in the female carrier heterozygotes. Features like atrophic rhinitis, nasal and aural myiasis, syndactyly, cleft lip and/or palate, mental retardation and immunodeficiency are uncommonly seen in this syndrome. We hereby report a case of hypohidrotic ectodermal dysplasia with unusual features of atrophic rhinitis and nasal myiasis. |
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Keywords: | Hypohidrotic ectodermal dysplasia Atrophic rhinitis Myiasis |
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