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Year : 1969  |  Volume : 35  |  Issue : 1  |  Page : 11--16

Congenital Ectodermal Defect


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N Sreedevi


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Two cases of congenital ectodermal defect are presented showing the typical facies, soft skin and feminine hair distribution. Skin biopsy from the forearm and axillae showed absence of sweat, sebaceous and apocrine structures. Sweat function test with bromo-phenol was negative as compared with a control patient. Clinically patients were uncomfortable in the warm weather for which they used to control their body temperature with frequent cold water soaks. Nasal examination showed associated chronic atrophic rhinitis. Axillary and pubic hair were absent and examination of genitalia showed hypospadias in case 1. Dental abnormalities seen in case 1. In the upper teeth, 8 deciduous teeth were present and lower teeth four in number, the two central being permanent and the other two deciduous. In case 2 all teeth were absent and patient had an artificial denture.






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Online since 15th March '04
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