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Amelo-Onycho-Hypohidrotic Syndrome

Last updated Sept. 11, 2021

Reviewed by: Lester Fahrner, MD

Approved by: Krish Tangella MD, MBA, FCAP

Amelo-Onycho-Hypohidrotic Syndrome forms a group of conditions of unknown cause that are marked by abnormal development of the teeth and nails, along-with decreased 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 forms a group of conditions of unknown cause that are marked by abnormal development of the teeth and nails, along-with decreased sweating
  • A treatment of the condition may include the use of tooth implants, topical agents, and drinking plenty of fluids. The prognosis of Amelo-Onycho-Hypohidrotic Syndrome may depend upon its severity but can be generally good

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

  • Amelo-Onycho-Hypohidrotic Syndrome is an extremely rare disorder 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)

Currently, the exact cause of Amelo-Onycho-Hypohidrotic Syndrome is not well-understood.

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

The signs and symptoms of Amelo-Onycho-Hypohidrotic Syndrome may vary from one individual to another and may include:

  • Tooth enamel abnormalities; yellow-brown discoloration of the teeth
  • Early or late eruption of teeth; reduced number of teeth
  • Decreased sweating
  • Abnormality of the fingernails and toenails
  • Thickening of the outer skin layers (hyperkeratosis)
  • Onycholysis, or the detachment of nail from nail bed
  • Dry skin 
  • Hair abnormalities including fine hair
  • Seborrheic dermatitis

How is Amelo-Onycho-Hypohidrotic Syndrome Diagnosed?

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

  • Physical examination of the individual and medical history evaluation
  • Assessment of the presenting signs and symptoms
  • Oral and dental examination
  • Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
  • Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
  • Skin biopsy, if necessary: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
  • Diagnostic tests to check for an underlying disorder or condition if present

A differential diagnosis may be necessary to eliminate other conditions that present similar signs and symptoms.

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 Amelo-Onycho-Hypohidrotic Syndrome?

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. Sometimes, dental implants may be needed as replacements, or other dental appliances may be used
  • Drinking plenty of water may be recommended
  • Topical applications may be prescribed for infected nails including appropriate nail care

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

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 unknown.

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. Due to a rarity of the condition, the prognosis may be only 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:

https://www.dovemed.com/healthy-living/skin-disorders/

What are some Useful Resources for Additional Information?


References and Information Sources used for the Article:


Helpful Peer-Reviewed Medical Articles:


Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: April 19, 2018
Last updated: Sept. 11, 2021