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

• Acrometageria
• Familial Acrogeria
• Gottron Syndrome

What is Acrogeria, Gottron type? (Definition/Background Information)

• Acrogeria, Gottron type is a premature aging syndrome. Characteristic signs include fragile, thin skin on the hands and feet. Other parts of the body (e.g., face, forearms, and lower legs) are variably affected
• It is generally considered to be a mild, non-progressive, congenital form of skin atrophy due to the loss of the fatty tissue directly under the skin
• Other symptoms reported in individual cases include small hands and feet, prominent veins on the chest, small stature, small jaw, premature senility, premature hair greying, endocrine disturbances, and cataracts
• Currently the cause of Acrogeria, Gottron type is unknown

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

Who gets Acrogeria, Gottron type? (Age and Sex Distribution)

• Acrogeria, Gottron type is a rare congenital disorder. The presentation of symptoms may occur following the birth of the child
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Acrogeria, Gottron type? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for Acrogeria, Gottron type

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 Acrogeria, Gottron type? (Etiology)

The exact cause of development of Acrogeria, Gottron type is presently unknown.

What are the Signs and Symptoms of Acrogeria, Gottron type?

The signs and symptoms of Acrogeria, Gottron type may include: (Based on the frequency of symptoms observed) 

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

• Fine hair
• Irregular hyperpigmentation
• Joint hyperflexibility
• Lipoatrophy
• Thin skin

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

• Convex nasal ridge 
• Excessive wrinkled skin 
• Intellectual disability 
• Micrognathia 
• Scoliosis
• Short foot 
• Small hand 
• Telangiectasia of the skin

Occasionally present symptoms in 5-29% of the cases:

• Skeletal dysplasia 
• Skin ulcer

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

How is Acrogeria, Gottron type Diagnosed?

Acrogeria, Gottron type 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 Acrogeria, Gottron type?

The complications of Acrogeria, Gottron type may include:

• Severe skin abnormalities
• Difficulty in walking
• Decreased quality of life

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

How is Acrogeria, Gottron type Treated?

There is no cure for Acrogeria, Gottron type, since it is a genetic condition. The treatment is usually given to manage the signs and symptoms and any complications that develops.

How can Acrogeria, Gottron type be Prevented?

Currently, Acrogeria, Gottron type may not be preventable, since it is a genetic disorder.

• Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help in understanding the risks better during pregnancy
• If there is a family history of the condition, then genetic counseling will help assess risks, before planning for a child
• Active research is currently being performed to explore the possibilities for treatment and prevention of inherited and acquired genetic disorders

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

What is the Prognosis of Acrogeria, Gottron type? (Outcomes/Resolutions)

• The prognosis of Acrogeria, Gottron type is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Acrogeria, Gottron type:

• Acrogeria, Gottron type is also known by the following names: Metageria

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

http://www.dovemed.com/diseases-conditions/rare-disorders/