Doyne honeycomb dystrophy

What causes Doyne honeycomb dystrophy and who is at risk? 

Doyne honeycomb dystrophy is caused by a problem with a gene called EFEMP1. Genes are the instructions our bodies use to make new proteins that keep our cells working.  

The EFEMP1 gene makes a protein that acts like a biological glue to hold eye cells together, ensuring they keep their shape and don’t tear. An error in this gene causes the protein to fold incorrectly and fail. This stops your eye cells from clearing waste, leading to the build-up of harmful fats and proteins. These deposits, called drusen, stop nutrients from getting to cells in the eye and prevent other waste from being removed from eye cells. Over time, some of these eye cells can get damaged and die, which is what causes your sight loss.   

Most people with Doyne honeycomb dystrophy only have gradual changes to their vision over the years. Not everyone with DHD will develop these changes, and your eye specialist will monitor you carefully for signs of damage.  

Sometimes people with DHD can develop cracks below the layer at the back of the eye called the retina. The retina is the light-sensitive tissue at the back of the eye, and the macula is the central part of the retina responsible for detailed vision. Abnormal new blood vessels can grow through these breaks from the choroid layer beneath the retina. These vessels may leak fluid or blood, causing damage and scarring of the macula, which can lead to rapid change in central vision. This condition is called choroidal neovascularisation (CNV). 

Although this might sound frightening, it is not very common. If you do develop CNV, you can have treatments to help stop the blood vessels from leaking and protect your eyesight. If you notice any sudden changes to your vision, contact an eye specialist straight away. Early treatment is important to preserve your sight. 

DHD is inherited, which means that the genes are passed down from your parents. You only need one parent to have the faulty gene. If either your mother or father has it, you will have a 50% chance (one in two) of also getting DHD. It affects men and women equally and usually affects both eyes. 

How to recognise early symptoms of Doyne honeycomb dystrophy 

The sight changes caused by Doyne honeycomb dystrophy can start slowly, so early signs are easy to miss. For some people, they can come on faster if they are caused by leaking blood vessels causing scarring.  

The first symptoms people notice are: 

• Vision becoming blurry 
• Blind spots 
• Straight lines suddenly looking distorted 
• Problems seeing details 
• Difficulty recognising faces 
• Problems adjusting when light levels change quickly (like going from the bright outdoors to a darker room) 

If you notice any of the above symptoms, seek help from an eye specialist.  

 
How Doyne honeycomb dystrophy is diagnosed: tests and what to expect 
  

If you have symptoms of Doyne honeycomb dystrophy, you will need to see an eye specialist. There are a few different tests they can do to diagnose you: 

OCT (Optical Coherence Tomography), an advanced 3D scanner that takes detailed images of your eyes. 

Fluorescein angiogram (FA) involves an injection of dye into your arm. The dye goes through your body, staining the tissues in the eye. A special camera is then used to take photographs of the eye. The dye stains will help eye specialists see the eye tissues in more detail so they can see any damage. 

Fundus Autofluorescence (FAF) shows the health of the eye by looking for waste product build-up. Lipofuscin (waste product) shows up under special lights and can tell your eye specialist more about your eye conditions. 

You may also be offered genetic testing and genetic counselling. This will help you understand more about how the gene is passed on and the risks to your family. This can help you make informed decisions about family planning and the risk to any children you have or plan to have. 

  
Managing Doyne honeycomb dystrophy: treatments, lifestyle changes, and support 
  

There is no cure for Doyne honeycomb dystrophy. If you have new blood vessels that are growing through cracks in your retina (choroidal neovascularisation), then you can access treatment. Medication to stop the growth of new blood vessels and slow the growth of existing ones is available. This is called Anti-VEGF medicine and it is given to you by injection into the eye.  

Having injections into the eye can be worrying for some people. Many people who are planning to have this treatment find our webpage on eye injections helpful to reduce their worries. If you still have questions, you can call our helpline on 0300 3030 111 – we will tell you more about what to expect from eye injections. 

How to reduce your risk of Doyne honeycomb dystrophy 

As Doyne honeycomb dystrophy is passed on through your mother or father, there isn’t anything you can do to reduce your risk.  

What you can do if you know you have a family history of DHD is: 

• Have regular eye check-ups to monitor your eye health. 
• Some people find that using an Amsler grid regularly helps them notice any changes in vision. By covering one and then the other eye, the grid will look wavy or there will be gaps in the lines if you are experiencing eye changes. Other people will notice changes when doing their usual everyday activities.   
• Stop smoking as this can worsen eye health generally. 
• Eat a healthy, balanced diet, with plenty of dark, leafy green vegetables, as these contain ingredients which keep eyes healthy. 
• Wear sunglasses or tinted lenses to reduce eye strain. 

Living with Doyne honeycomb dystrophy  
 

Many people with central vision loss caused by DHD find life presents new challenges as symptoms develop. Sensitivity to light can bring pain and discomfort, and there might be psychological changes too. Getting the right support, as well as the right equipment, can help.   

Practical changes for living with DHD 

You can keep living a healthy, active life by making a few small changes. These can include: 

Using low vision aids and assistive technology to help you complete daily tasks more easily.  

Adjusting the settings on e-readers, tablets, phones and computer screens to larger text, or using screen readers. 

Redesigning the lighting in your home – use our lighting guide for helpful tips and ideas. 

Your mental health when you have DHD 

Even though your family members might already be living with symptoms of DHD, finding out you have it too can come as a shock. This can have a big impact on your emotional health and cause you to feel very low.  

Getting the right support is very important for your mental wellbeing. As your family members might also have the condition, talking to them can help. They might have some tips and tricks they can recommend or can just listen to your worries.  

Counselling can also be helpful. The Macular Society can refer you for counselling, or you can access it through the NHS by speaking to your GP.  

Working when you have DHD 

As Doyne honeycomb dystrophy affects people in early-to-mid adulthood, most people will still be of working age. Working with an eye condition can be difficult, but there are 'reasonable adjustments’ your employer can make to help you continue to succeed in the workplace. 

These can include: 

• A redesign of the lighting in the workplace to make it bright and get rid of glare 
• More time to complete tasks 
• Screen reading software 
• Large print 
• Use of magnifiers and other low vision aids 

For more advice, support and information, call the Macular Society helpline on 0300 3030 111.

You don’t have to face your challenges alone. 

Current research and developments for Doyne honeycomb dystrophy 
  

Research is currently focusing on new treatments. The Macular Society has funded a study investigating whether gene therapy can ‘turn off’ the faulty genes involved in DHD. Dr van der Spuy’s research has now concluded with very promising results. You can learn more and watch a video about the research for more information. 

Discover more support 
 

Whatever your worries about living with Doyne honeycomb dystrophy, the Macular Society is here for you. We provide the following services, free of charge: 

Our Macular Society Helpline is open Monday to Friday from 9am to 5pm.  Call us on 0300 3030 111 to speak to a helpful and compassionate advisor.  

Services for anyone who is finding life tough and who have noticed a decline in their mental health. We offer referrals for counselling and we can even find you a buddy through our befriending service. There’s also a network of support groups across the UK. 

We have lots of guidance on subjects like employment, going out, redesigning the lighting in your home and tips on using low vision aids. 

We also have a Working age and Young people Facebook page – visit us there to talk to our community, introduce yourself and discuss your diagnosis. 

FAQs about Doyne honeycomb dystrophy 

What is Doyne honeycomb dystrophy (DHD)? 

DHD is a rare eye condition that is caused by a build-up of waste products in the eye. These waste products stop nutrition from getting to eye cells, which then die. The death of these cells causes vision issues. 

Will Doyne honeycomb dystrophy cause me to go blind? 

Your central vision will be affected by DHD but you should still have the ability to see around the sides of your eyes.

When will symptoms of DHD start? 

People usually start to notice symptoms around 20 to 30 years old. Some people might experience initial problems when they are in their teens. It is different for everyone, even within families. 

If my mum or dad has DHD, will I get it? 

You only need one parent to have DHD for you to get it. If they do, you have a 50% chance of getting it too. You will also have a 50% chance of passing it to your child. 

Can DHD get worse over time? 

It can get worse over time but some people will have a slower progression of symptoms than others. 

What treatments are there for Doyne honeycomb dystrophy? 

There is no cure for DHD but you might benefit from eye injections, depending on what your symptoms are. 

Last review date: 08 2026

Next review date: 08 2028