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Amelo-Onycho-Hypohidrotic Syndrome

Last updated April 19, 2018

Approved by: Krish Tangella MD, MBA, FCAP

Amelo-Onycho-Hypohidrotic Syndrome encompasses a group of disorders, characterized by abnormalities in teeth, nails, and sweating.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Ameloonychohypohidrotic Syndrome
  • Hypocalcified-Hypoplastic Enamel, Onycholysis with Subungual Hyperkeratosis, and Hypohidrosis

What is Amelo-Onycho-Hypohidrotic Syndrome? (Definition/Background Information)

  • Amelo-Onycho-Hypohidrotic Syndrome encompasses a group of disorders, characterized by abnormalities in teeth, nails, and sweating
  • The signs and symptoms of Amelo-Onycho-Hypohidrotic Syndrome include discolored teeth, abnormal enamel, under-developed nails, separation of nails from nail bed, reduced sweating and dry skin, among others
  • The exact cause of this disorder is not known at the present time

Who gets Amelo-Onycho-Hypohidrotic Syndrome? (Age and Sex Distribution)

  • Amelo-Onycho-Hypohidrotic Syndrome is rare and of unknown prevalence
  • Both males and females may be affected
  • Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Amelo-Onycho-Hypohidrotic Syndrome? (Predisposing Factors)

  • Currently, no risk factors have been clearly identified for Amelo-Onycho-Hypohidrotic Syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Amelo-Onycho-Hypohidrotic Syndrome? (Etiology)

The exact cause of Amelo-Onycho-Hypohidrotic Syndrome is presently not known.

What are the Signs and Symptoms of Amelo-Onycho-Hypohidrotic Syndrome?

The signs and symptoms of Amelo-Onycho-Hypohidrotic Syndrome may include:

Very frequently present symptoms in 80-99% of the cases:

  • Abnormality of dental enamel
  • Abnormality of the fingernails
  • Hyperkeratosis
  • Hypohidrosis
  • Hypoplastic toenails
  • Onycholysis
  • Yellow-brown discoloration of the teeth

Frequently present symptoms in 30-79% of the cases:

  • Abnormality of dental morphology 
  • Advanced eruption of teeth 
  • Delayed eruption of teeth
  • Dry skin 
  • Fine hair
  • Reduced number of teeth

Occasionally present symptoms in 5-29% of the cases: Everted lower lip vermilion

Other signs and symptoms may include:

  • Abnormality of the hair
  • Marked delay in eruption of permanent teeth
  • Seborrheic dermatitis

(Source: Ameloonychohypohidrotic Syndrome; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

How is Septo-Optic Dysplasia Spectrum Diagnosed?

Amelo-Onycho-Hypohidrotic Syndrome is diagnosed on the basis of the following information:

  • Complete physical examination
  • Thorough medical history evaluation
  • Assessment of signs and symptoms
  • Laboratory tests
  • Imaging studies
  • Biopsy studies, if necessary

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis

What are the possible Complications of Septo-Optic Dysplasia Spectrum?

The complications of Amelo-Onycho-Hypohidrotic Syndrome may include:

  • Tooth decay due to thin enamel
  • Problems with chewing food
  • Brittle nails
  • Severe infection of the nail bed leading to nail loss
  • Issues with temperature control in the body, due to reduced sweating 

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is Amelo-Onycho-Hypohidrotic Syndrome Treated?

Treatment for Amelo-Onycho-Hypohidrotic Syndrome may be given to manage the signs and symptoms, which may include:

  • In case of extremely thin teeth, a crown can be placed to preserve the teeth and prevent further stress and damage. Crowns may also help combat the problems of increased tooth sensitivity
  • In case of severe damage to the teeth, it may be necessary to completely remove the teeth; or, implants may be needed
  • Drinking plenty of water may be recommended
  • Topical applications may be prescribed for infected nails

How can Amelo-Onycho-Hypohidrotic Syndrome be Prevented?

  • Currently, no methods or guidelines exist for the prevention of Amelo-Onycho-Hypohidrotic Syndrome, since the cause and risk factors for the syndrome are not known
  • Active research is currently being performed to explore the possibilities for treatment and prevention of such disorders

Regular medical screening at periodic intervals with tests and physical examinations are recommended.

What is the Prognosis of Amelo-Onycho-Hypohidrotic Syndrome? (Outcomes/Resolutions)

  • The prognosis of Amelo-Onycho-Hypohidrotic Syndrome is dependent upon the severity of the signs and symptoms and associated complications, if any
  • Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Amelo-Onycho-Hypohidrotic Syndrome:

The following DoveMed website link is a useful resource for additional information:


What are some Useful Resources for Additional Information?

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Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: April 19, 2018
Last updated: April 19, 2018