Clinical History.—A 4-year-old boy has had poor tolerance to heat and recurrent episodes of fever since birth. He has been hospitalized six times in the past three years for repeated respiratory infections. Roentgenograms of the paranasal sinuses were requested to rule out sinusitis (Fig 1 and 2).
Denouement and Discussion
Congenital Anhidrotic Ectodermal Dysplasia
This diagnosis can be made at birth, roentgenographically, since all of the deciduous teeth are calcified by this time and can be identified on roentgenograms of the mandible (Fig 1 and 2). The diagnosis of anhidrotic ectodermal dysplasia was made on this child by 1 year of age based on the typical findings2 of hypotrichosis, a peculiar facial appearance, recurrent febrile episodes, absence of sweat, and failure of tooth eruption. Two forms of ectodermal dysplasia have been described, hidrotic and anhidrotic, based on the presence or absence of exocrine sweat glands. Recently, involvement of
Gwinn JL, Lee FA. Radiological Case of the Month. Am J Dis Child. 1974;128(2):215-216. doi:10.1001/archpedi.1974.02110270089018