Diagnosis
- Prenatal testing is available in some centers.
- History
- Family history
- Onset of symptoms (usually at birth- recurrent unexplained fevers)
- Clinical
- Depends on the organs being affected
- Sweat glands
- Absence/reduced sweat gland production results in disrupted thermoregulation which can lead to abnormally high temperatures in children.
- Seizures and neurological problems occur as a sequalae.
- Hair
- Thinning and sparsity of the hair present in the scalp and body.
- The facial, axillary and pubic hairs are usually spared.
- Nails
- Fingernail and toenail abnormalities including thinning, ridging, discoloration, brittle nails, or absence of nails.
- Teeth
- Disfigurement in tooth shape, including pointed/peg shaped teeth. Teeth may also be missing
- Other symptoms:
- Skin: dryness, pigmentation, thickening leading to cracking, bleeding, and infections
- Eyes: Keratoconjunctivitis sicca due to lack of tear production. Cataracts and visual defects can occur.
- Mucous membranes: foul smelling nasal discharge and respiratory infections due to decreased synthesis of normal protective secretions.
- Cleft lip/palate
- Histopathology
- Skin biopsy shows reduced numbers of sweat glands
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