Introduction
As time passes, our bodies naturally get older. Premature ageing is when the usual signs of getting older happen earlier than they should. It's when your body appears older than it really is.
Definition of premature ageing
Premature ageing syndromes, also called progeria, are very rare inherited conditions. There are two types: Hutchinson-Gilford syndrome and Werner syndrome. In both of these conditions, the skin goes through changes that show ageing happening too soon. These changes include:
- Skin becoming thinner and less elastic
- Loss of fat under the skin
- Developing wrinkles
- Hair turning grey and falling out
- Nails becoming yellow, thin, brittle, or curved
- Skin losing its normal colour
- Skin developing sores
These changes usually occur as people get older, but in progeria, they happen much earlier.
The word "progeria" comes from the Greek word "geras," which means "old age." The most common type of progeria is called Hutchinson-Gilford progeria syndrome, or HGPS. Doctors named it after Dr. Jonathan Hutchinson and Dr. Hastings Gilford, who first described the disease in the late 1800s.1
Importance of understanding premature ageing
Premature ageing is a condition that is always deadly. On average, people with progeria pass away at around 14.5 years old, although some may live into their early 20s. A drug called lonafarnib has been found to slow down the progression of the disease.
The main reason for death in progeria is a severe form of heart disease called atherosclerosis. This is the same heart disease that affects many older adults, but in progeria, it happens much earlier. Atherosclerosis occurs when a substance called plaque builds up inside the walls of your arteries. This makes your arteries less flexible and more rigid.2
How often does premature ageing occur?
Progeria or premature ageing is an incredibly uncommon condition. It only occurs in 1 out of every 4 million births worldwide. Right now, approximately 400 children and young adults in the world have progeria.3
Who can have premature ageing?
Premature ageing or progeria is a rare genetic condition that can affect anyone. Usually, it happens because of a new genetic change (mutation) in a person's DNA, meaning it's not something that runs in the family.3
Causes of premature ageing
Genetics
Premature ageing or progeria is caused by a single gene called lamin A (LMNA). This gene is responsible for making a protein that helps hold the centre of a cell, called the nucleus, together. When there's a change in the LMNA gene, it produces a faulty protein called progerin. Progerin makes cells unstable and seems to be the reason behind the ageing effects seen in progeria.
The genetic change that leads to progeria isn't something that families usually pass down to their children. Instead, it typically happens unexpectedly by chance.4
Lifestyle factors
Often, appearing older than your age is within your control and can be prevented. This is known as extrinsic ageing.
- Smoking: Smoking is harmful because the chemicals in nicotine can damage your body's cells. They break down collagen and elastic fibres in your skin, leading to sagging, wrinkles, and a ‘worn-out’ appearance.
- Unhealthy diet: Some studies suggest that diets high in sugar or processed carbs might make you age faster. But if you eat lots of fruits and vegetables, it can help keep your skin healthy and prevent early ageing.
- Alcohol: Drinking too much alcohol can dry out and harm your skin over time, causing signs of premature ageing.
- Poor sleep: Not getting enough good-quality sleep can speed up the ageing of your cells.
- Stress: When you're stressed, your brain releases a hormone called cortisol, which can harm your skin by blocking substances that keep it looking plump and youthful, like hyaluronan synthase and collagen.5
Environmental factors
Sunlight can harm your skin a lot. One type of light from the sun, called ultraviolet (UV) light, makes your skin age quicker than it should. This is called photoaging, and it's responsible for 90% of the changes you can see on your skin. UV light hurts your skin cells and causes early ageing, like getting age spots. It also raises the chance of getting skin cancer.
There are other kinds of light, like high-energy visible (HEV) and infrared light, that cause the remaining 10% of skin changes. HEV light, also known as blue light, comes from the sun and things like smartphones. Infrared light is invisible, but it feels warm. These types of light don't make skin cancer more likely, but they do affect how bouncy your skin is and its collagen level.5
Signs and symptoms of premature ageing
Kids with progeria typically look normal when they're born. However, around their first year, they might start to show signs like slow growth, losing fat under their skin, and hair falling out.
Sadly, most children with progeria pass away because of heart problems or strokes. On average, they live to about 15 years old. Some might not make it to that age, while a few may live a bit longer, maybe even to around 20 years old.
In that first year, you might notice that your child isn't growing as quickly as other kids. But their ability to move and their intelligence aren't affected.6
Distinctive appearance in premature ageing
- Slowed growth and poor weight gain, resulting in below-average height and weight.
- A lack of fat beneath the skin.
- A relatively large head compared to the face.
- A small jaw, chin, and mouth, along with thin lips.
- A thin nose with a slight hook at the end, resembling a bird's beak.
- Large eyes and eyelids that don't close fully.
- Hair loss, including the eyelashes and eyebrows.
- Thin, spotty, and wrinkled skin.
- Visible veins beneath the skin.
- A high-pitched voice.
- Premature ageing.6
Health issues and premature ageing
Premature ageing comes with various problems:
- Serious heart and blood vessel issues that get worse over time.
- Skin that becomes hard and tight.
- Teeth that take longer to form and may not look normal.
- Some trouble with hearing.
- Losing the fat under the skin and muscle.
- Growth and bone development issues.
- Common problems, like stiff joints.
- A hip joint being in the wrong place is called hip dislocation.
- Dental complications.
- Not going through puberty like other people do.
- The Body doesn't respond well to insulin made by the pancreas, which is called insulin resistance.6
Prevention and management of premature ageing
To stop premature ageing and prevent it from getting worse, you can do these things:
- Protect your skin from the sun by using sunscreen, wearing protective clothing, and avoiding tanning beds.
- Quit smoking if you smoke, and ask your doctor for help if you need it.
- Eat more fruits and vegetables, and avoid too much sugar and processed foods.
- Cut down on alcohol because it can make your skin age faster.
- Exercise regularly to keep your body healthy.
- Take care of your skin by cleaning it daily, avoiding harsh products, and using moisturiser.
- Find ways to reduce stress, like meditation or exercise.
- Get enough good-quality sleep because not sleeping enough can make your cells age faster.
If you already have signs of premature ageing and want to make them look better, you can try these treatments:
- Dermal fillers
- Facelift
- Procedures like facial resurfacing and rejuvenation
- Laser skin resurfacing
Keep in mind that these treatments can make your appearance better, but they won't fix the underlying cause of premature ageing.6
Summary
- Premature aging, also known as progeria, is a rare condition where the usual signs of ageing happen much earlier than normal.
- Premature ageing is caused by genetic mutations and can affect anyone, typically without a family history of the disorder.
- The condition results in distinctive physical characteristics such as thin skin, hair loss, and cardiovascular issues, and it often leads to a shortened lifespan, with heart problems being a major cause of death.
- Factors like sun exposure, smoking, an unhealthy diet, alcohol consumption, stress, and poor sleep can contribute to premature ageing, emphasising the importance of healthy lifestyle choices.
- While there are cosmetic treatments available to improve the appearance of premature ageing, they do not address the root causes of the condition. Prevention through a healthy lifestyle is crucial to minimising premature ageing effects.
References
- Progeria - Symptoms and causes. Mayo Clinic, https://www.mayoclinic.org/diseases-conditions/progeria/symptoms-causes/syc-20356038 (accessed 22 September 2023).
- Progeria (Hutchinson-Gilford Progeria Syndrome — HGPS): Symptoms & Causes. Cleveland Clinic, https://my.clevelandclinic.org/health/diseases/17850-progeria (accessed 22 September 2023).
- Sharma V, Shukla R. Progeria: A Rare Genetic Syndrome. Indian J Clin Biochem 2020; 35: 3–7.
- Gonzalo S, Kreienkamp R, Askjaer P. Hutchinson-Gilford Progeria Syndrome: A premature aging disease caused by LMNA gene mutations. Ageing Res Rev 2017; 33: 18–29.
- Gordon LB, Brown WT, Collins FS. Hutchinson-Gilford Progeria Syndrome. In: Adam MP, Mirzaa GM, Pagon RA, et al. (eds) GeneReviews®. Seattle (WA): University of Washington, Seattle, http://www.ncbi.nlm.nih.gov/books/NBK1121/ (1993, accessed 22 September 2023).
- Macicior J, Marcos-Ramiro B, Ortega-Gutiérrez S. Small-Molecule Therapeutic Perspectives for the Treatment of Progeria. Int J Mol Sci 2021; 22: 7190.