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Adam MP, Feldman J, Mirzaa GM, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2024.

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Table 5.

Treatment of Manifestations in Individuals with Hypohidrotic Ectodermal Dysplasia

Manifestation/ConcernTreatmentConsiderations/Other
Hypotrichosis Wigs or special hair care formulas & techniques to manage sparse, dry hair1 report describes a child w/HED & alopecia who was treated w/topical minoxidil to scalp & had resultant hair growth. 1
Hypohidrosis
  • During hot weather: access to adequate water supply & cool environment (e.g., air conditioning, wet T-shirt, &/or spray bottle of water) 2
  • Skin care products for mgmt of dry skin, eczema, & rashes assoc w/certain outdoor exposures (e.g., swimming)
Some persons may benefit from cooling vests.
Hypodontia
  • Dental treatment, ranging from simple restorations to dentures, should begin at early age.
  • Treatments incl restorations, bonding of conical teeth, dental implants, &/or dentures.
  • Orthodontics may be necessary.
  • Dietary counseling for those persons who have trouble chewing & swallowing despite adequate dental care
  • Bonding of conical teeth improves aesthetics & chewing ability.
  • Dental implants in anterior portion of mandibular arch have proven successful only in children age ≥7 yrs. 3
  • Children w/HED typically need dental prostheses replaced every 2.5 yrs.
  • Dental implants in adults can support aesthetic & functional dentition.
Hyposalivation
  • Therapeutics (e.g., saliva substitutes) directed at maintaining oral lubrication & to ↓ risk of dental caries
  • Fluoride treatments as recommended by dentist
  • Consider other dental caries preventive approaches such as pit & fissure sealants.
Solidified nasal &
aural secretions
  • Mgmt per ENT physician
  • Removal of nasal & aural concretions w/suction devices or forceps
  • Humidification of ambient air to prevent their formation
Dry eyes Lubrication eye drops
Respiratory manifestations
  • Mgmt of recurrent respiratory infections & asthma per primary care provider
  • Referral to allergist &/or pulmonologist as needed for more significant respiratory manifestations
1.
2.

Affected individuals learn to control their exposure to heat and to minimize its consequences, but special situations may arise in which intervention by physicians and families is helpful. For example, a physician may have to prescribe an air conditioner before a school district complies, or parents may have to advocate for children who need to carry liquids into areas where they are prohibited.

3.

From: Hypohidrotic Ectodermal Dysplasia

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