A rare cause of fever in the emergency department: Anhidrotic ectodermal dysplasia

Abstract

Ectodermal dysplasia is a rare disease that present with: hypotrichosis, hypodontia, and typically absence of eccrine sweating. It consists of two basic forms; anhidrotic and hidrotic. In anhidrotic ectodermal dysplasia, the body's thermoregulatory mechanism is impaired due to lack of sweat glands. These patients may therefore present to the emergency department with hyperthermia. This report describes a patient with anhidrotic ectodermal dysplasia brought to the emergency department in an unconscious state and with a fever of 41°C. This rare disease should be kept in mind in patients presented to ED with an unconscious state due to hyperthermia. Typical clinical appearance is a useful clue for the diagnosis. Proper antipyretic therapy is sufficient for the treatment.