ABSTRACT
Anhidrotic ectodermal dysplasia is inherited as an X linked recessive trait. This disor der is characterized by hypotrichosis, hypodontia and hypohidrosis. The diagnosis is often delayed until the first or second year of life, after repeated episodes of potentially damaging high fever. In the newborn period, the diagnosis is more difficult, but early diagnosis is of importance in ensuring that the appropriate enivironment and medical measures be taken to avoid uncontrolled hyperthermia. We have experienced a case of anhidrotic ectoclermal dysplasia in an8-day-old male patient who showed charecteristic features including hypotrichosis, peeling or scaling of the skin, recurrent fever and a characteristic face. A skin biopsy from the right palm revealed no sweat gland strutures. A brief rview with related literature is also presented.