What Is Werner Syndrome?

Werner syndrome is a premature aging syndrome. It's similar to Hutchinson-Gilford syndrome, also known as child’s progeria or Benjamin Button disease (nicknamed for the Brad Pitt movie where his character ages in reverse).

Both syndromes are inherited, have similar symptoms, and cause a person to look as if they’re aging prematurely. The main difference between the syndromes is the time at which symptoms appear.

While progeria presents in children, people with Werner syndrome may not know they have the disease until their teens or adulthood.

Here is an overview of Werner syndrome, including its symptoms, how it is diagnosed, and what can be done to manage the condition.

The WRN Gene

The gene that causes Werner syndrome—the WRN gene—controls Werner protein production. This protein is thought to have a role in DNA repair and maintenance. A mutation in this gene can cause Werner syndrome. Experts believe the damage causes Werner syndrome symptoms, but they are unsure of exactly how it occurs.

Werner syndrome affects approximately one in 1 million people. The condition is more common in Japan than in other parts of the world. The condition affects males and females equally.

A child with progeria playing with puzzle pieces.

Ilknur Hicret/Getty

Werner Syndrome Symptoms

A person with Werner syndrome will exhibit signs of premature aging such as:

  • Thinning skin
  • Loss of skin firmness
  • Fat loss in the upper layers of the skin
  • Hair loss
  • Wrinkling
  • Distortion and discoloration of the nails
  • Hair greying
  • Pigmentation issues
  • Ulcers

The signs of early aging usually appear during a person’s 30s or 40s but can also start earlier.

People with Werner syndrome may:

  • Have a small jaw
  • Have a sunken appearance
  • Be short
  • Lack strength
  • Have a high-pitched voice
  • Develop eye issues (such as cataracts)
  • Develop endocrine disorders
  • Have dental issues
  • Have a thick trunk and thin limbs

Causes

Werner syndrome is an inherited condition, meaning a person is born with it. A person must inherit the mutated WRN gene from both parents for Werner syndrome to occur.

If both parents carry the mutated gene, it does not mean all of their children will be born with the disease—one in four children will usually have the disorder.

Diagnosis

Unlike progeria, Werner syndrome is not diagnosed at birth. That's why it is referred to as progeria of adults. It's usually diagnosed in a person’s 30s or 40s but can be diagnosed as early as age 15.

Most people start having symptoms, such as hair greying and alopecia, early on. People with Werner syndrome can have other characteristics in childhood and adolescence including:

  • Slow growth rate
  • Short stature
  • Low weight compared to their height
  • Lack of pubic hair, breasts, and menstruation
  • Abnormally small penis and testes

The only way to confirm a diagnosis of Werner syndrome is through molecular gene testing.

Other Related Diseases

Some diseases that have similar symptoms to Werner syndrome include:

  • Hutchinson-Gilford syndrome
  • De Barsy syndrome
  • Gottron’s syndrome
  • Storm syndrome
  • Mulvihill-Smith syndrome
  • Rothmund-Thomson syndrome

Treatment

There is no treatment for Werner syndrome. The focus is on managing symptoms and monitoring for complications such as tumors, heart disease, and diabetes.

People with Werner syndrome need a team of medical specialists to help them manage the disease. Examples of conditions that a person might develop include:

  • Type II diabetes (which is usually mild in people with Wener syndrome and is easy to treat)
  • Eye issues such as cataracts (which may require surgery or corrective lenses)
  • Cancer (which can be treated the same as it would be in a person without Werner's syndrome)

Prognosis 

People with Werner syndrome are at higher risk for cancer and cardiovascular disease. Heart disease occurs because of the loss of elasticity and thickening of the arteries.

Cancers common in people with Werner syndrome include:

It's important that doctors monitor a person for complications of Werner syndrome, which often show up in a person's 40s and 50s and can be life-threatening.

Coping 

People with Werner syndrome can manage their symptoms and avoid health complications with the help of a team of specialists.

If a person with Werner syndrome wants to conceive, experts recommend they undergo genetic counseling.

Preimplantation genetic diagnosis (PGD) involves testing eggs for the genetic mutation before implantation. While this can be an option for people with Werner syndrome who are undergoing in vitro fertilization (IVF), the cost can be a barrier.

Summary

Werner syndrome is a rare disease that is similar to progeria but usually diagnosed in teens and adults instead of babies and kids. It causes a person to age faster than normal. The condition is caused by a genetic mutation. There is no specific treatment for Werner's syndrome, but a team of specialists can help people manage the condition and avoid health complications.

A Word From Verywell

If you or a loved one has been diagnosed with Werner syndrome, know that while there are still many unknowns about the condition, it can be managed.

A team of medical specialists who are familiar with the syndrome will help you cope with its symptoms and avoid complications. They can also guide you through some decisions that you might be considering in your personal life that could be affected by Werner syndrome, such as wanting to have children.

Frequently Asked Questions 

What genetic mutation causes Werner syndrome?

A mutation in the WRN gene causes Werner syndrome, but experts still do not understand how the mutation causes the symptoms characteristic of the condition.

How common is premature aging disease?

Premature aging diseases are rare. Werner syndrome affects about one in 1 million people worldwide, while Hutchinson-Gilford syndrome affects approximately one in 8 million children worldwide.

What is the link between Werner syndrome and cancer?

People with Werner syndrome are likely to develop cancer as a complication of the disease, likely because of the rapid aging they experience. Many cancers tend to develop later in life, but for people with premature aging diseases like Werner syndrome, cancers may appear earlier.

How does Werner syndrome change your appearance?

People with Werner syndrome start to get grey hair sooner than people who do not have the condition, and they lose skin elasticity and wrinkle prematurely.

People with Werner syndrome are usually shorter than average. As they get older, they develop a “pinched” appearance in the face. Their skin and nails become discolored, and they will experience hair loss all over the body. Some people with the condition have been described as having a "bird-like" appearance.

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6 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  1. U.S. National Library of Medicine. Werner syndrome. Updated April 7, 2021.

  2. DermNet. Premature ageing syndromes.

  3. National Organization for Rare Disorders. Werner syndrome. Updated June 18, 2018.

  4. Genetic and Rare Diseases Information Center. Werner syndrome.

  5. CancerNet. Werner syndrome.

  6. Shamanna RA, et al. Recent advances in understanding Werner syndrome. F1000Res. 2017;6: 1779. doi:10.12688/f1000research.12110.1