Anhidrosis (Congenital Anhidrotic Ectodermal Dysplasia)

Rare: 1:125,000,000

Clusters in Japan and Israel

Impaired thermoregulation (risk of fatal hyperpyrexia in infants)

Postop chest infections

Absence of sweat leads to impaired thermoregulation.

Insensitivity to superficial and deep painful stimuli with intact tactile perception; still require considerable amounts of inhalational or IV anesthetics to maintain hemodynamic stability and obtund stress response to airway manipulation.

Innervation of the eccrine sweat glands is lacking; heat loss by evaporation is impaired.

Absent mucous glands from resp tract and esophagus; frequent resp infections.

Partial or complete absence of teeth.

Hypotrichosis (absent hair).

Self-mutilating behavior and mental retardation.

Characteristic facies: Prominent supraorbital ridges, depressed bridge and root of nose, large deformed ears, thick lips, underdeveloped maxilla and mandible.