ABSTRACT

The ectodermal displasias are a heterogeneous group of disorders with a primary defect in hair, teeth, nail or sweat gland function. Hypohydrotic (anhydrotic) ectodermal dysplasia (HED) is one of the distinctive syndromes and may cause considerably difficulty during the first year of life. The most serious disturbance is the absence or diminution of sweating, due to rudimentary or absent ecrine sweat glands, which results in marked heat intolerance and episodes of hyperpyrexia. In this report, a neonate hospitalized with fever of unknown origin and diagnozed as HED is presented.