Ocular mucous membrane pemphigoid (ocular cicatricial pemphigoid)

What is ocular mucous membrane pemphigoid?

Ocular mucous membrane pemphigoid (also known as OcMMP, OMMP, or ocular cicatricial pemphigoid) is a chronic, severe type of conjunctivitis that leads to scarring. It is part of a group of autoimmune conditions called mucous membrane pemphigoid (MMP) - ocular means it affects the eyes. 

MMP can occur in a single site in the body or across several areas. 

What causes ocular mucous membrane pemphigoid?

Ocular mucous membrane pemphigoid is an autoimmune disease - this means the body’s immune system is mistakenly attacking healthy tissue. Our immune systems are designed to produce antibodies to help fight infections, but in autoimmune disease, the immune system begins to produce antibodies which attack normal tissue (known as autoantibodies). 

Mucous membrane pemphigoid affects the mucous membranes found in areas of the body that are naturally moist, such as inside the mouth, throat, and nose, as well as the eyes, genitals and areas of the skin.

What are the signs and symptoms of ocular mucous membrane pemphigoid?

Common signs and symptoms include:

• Conjunctivitis: This is usually one of the first symptoms, and can be mild or severe. Conjunctivitis means inflammation of the conjunctiva, the membrane that lines the eyelids and covers the white of the eye. Eyes and eyelids become red and inflamed and may also look and feel sticky, irritated, sore, or painful. If it does not improve with antibiotics, or the condition keeps returning despite using antibiotics, this could be a sign of MMP.
• Scarring: Persistent conjunctival inflammation leads to scarring, which can happen quite quickly if the inflammation is severe. The scarring may not be visible to the naked eye.
• Drooping eyelids: as the condition progresses, the gaps between the eyelids and eyeball (the conjunctival fornix) can become smaller, causing lids to appear more droopy or closer together, with less of a gap between them.
• Eyelids begin to turn inwards: known as trichiasis. This means the eyelashes can start to scratch the eyeballs. The scratching can damage the cornea, the clear window of the eye, which can then become vulnerable to infection.
• Dryness of the eyes: as tubes that come from the glands and drain tears become blocked, they can become less effective, resulting in the eyes becoming dry. This can worsen problems with the eyes.
• Blepharitis: the eyelid margin glands, situated along the edges of the eyelids near the eyelashes, can become blocked, resulting in further swelling, inflammation and irritation.

Is ocular mucous membrane pemphigoid serious?

Ocular mucous membrane pemphigoid is a chronic and often severe condition with no cure. The scarring associated with the condition can lead to sight loss, which may be severe in some cases, resulting in irreversible blindness - so early diagnosis is essential to avoid scarring and sight loss.

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How is ocular mucous membrane pemphigoid diagnosed?

If you have MMP symptoms in other areas of the body, you would probably be referred by your GP to a dermatologist (skin specialist), for a biopsy (small tissue sample) of the affected areas. If your symptoms are only in your eyes, an ophthalmologist (eye doctor) would do a biopsy of the conjunctiva. 

Before you have the biopsy, anaesthetic drops are put into both eyes so that it doesn't hurt. Frequently, a biopsy is also taken from the lining of the mouth, which is the most common site for MMP. The samples are sent off to specialist labs to be analysed. The tissue is tested against antibodies - this is called the direct immunofluorescence test (DIF).

A blood sample is also taken. This is tested for the presence of autoantibodies (serology testing or indirect immunofluorescence (IIF)).

How long it takes to get the results can vary between hospitals, but whoever carries out the tests should be able to advise on when you’re likely to receive results. If the results show you have ocular mucous membrane pemphigoid, you will usually receive a letter or telephone call arranging a follow-up appointment to discuss treatment options and any further examinations.

You may need further examinations if the test proves negative. When the eyes are the only site affected by MMP, sometimes a test result can be negative even when you have the condition.

Because OcMMP is rare, and the symptoms can seem similar to other conditions, it can be hard to diagnose.

What are the treatments for ocular mucous membrane pemphigoid?

There is no cure for ocular mucous membrane pemphigoid. However, treatments can make a big difference to your comfort levels and quality of life, as well as slowing down the progression of the condition. 

As with other autoimmune diseases, treatment may also help the condition to go into ‘remission’ – meaning symptoms stop completely or reduce to a much milder level. It is important that treatment for ocular mucous membrane pemphigoid starts as soon as possible, as it is much easier to prevent severe scarring from developing than treating it once it has occurred.

Treatment will depend on your specific condition, but some of the main treatment include:

• Immunosuppressive drugs: These are used to treat various autoimmune conditions, often when symptoms are fairly severe. They are the most common treatment for MMP and are usually taken as tablets. There are different varieties of immunosuppressive drugs – including azathioprine, mycophenolate and dapsone. The aim is to deliver a dose adequate to stop the disease whilst enabling the immune system to continue protecting you from infection.
• Biological treatments: These drugs may be an option when ocular mucous membrane pemphigoid is particularly severe. They also work by altering the immune system but are designed to be more targeted and ‘block’ specific processes involved in the disease.
• Intravenous immunoglobulin (IViG): This treatment is administered via a drip and is designed to replace the antibodies in a your system.
• Surgery: If severe scarring has caused the eyelids to turn inwards, corrective surgery can sometimes help. This may also be performed to remove eyelashes that can no longer be easily removed with tweezers (to prevent them from scratching the surface of the eye).

What can help ocular mucous membrane pemphigoid?

Eye drop and ointment treatments can help to relieve your symptoms, though they are usually not effective as a treatment on their own. 

Warming and cleaning your eyelids regularly can help manage ocular mucous membrane pemphigoid. Follow the simple process below:

• Apply warm compresses for 10 minutes - either a hot flannel soaked in boiled water that is cooled until it is comfortably warm, or you can buy an eyelid warming device or mask.
• Gently massage the upper and lower eyelids with your ring finger, moving in a circular motion to liquefy the oils.
• Gently express the oils out of the glands by rolling a cotton wool bud towards the eyelid margin and the lashes.
• Prepare a cleaning solution with half a teaspoon of bicarbonate of soda per half a pint of boiled and cooled water. Dip a cotton wool bud into the cleaning solution. Gently clean the eyelid margin just behind the roots of the lashes. Use a clean cotton wool bud for each eyelid. You can also buy eyelid wipes from chemists, opticians or online.

Living with a chronic condition like ocular mucous membrane pemphigoid can sometimes be challenging.

While treatments can be very effective, side effects can also occur, so it is important to discuss these with your doctor and attend regular follow-ups so things can be monitored.

It’s important to be aware that all chronic health conditions can also take a toll emotionally and mentally. Talking to your GP about this is a good idea if you are finding things tough and talking to your loved ones. Explaining what’s happening to your employer can also be very helpful, so they understand what you are dealing with and can support you as needed.

What research is there into ocular mucous membrane pemphigoid?

Fight for Sight’s goal is to help further the understanding of ocular mucous membrane pemphigoid and use this knowledge to develop prevention techniques and better treatments. There are ongoing research efforts to better understand the genetic and environmental factors involved in developing OcMMP and to find more effective treatments to improve patients' quality of life.

Fight for Sight's Research Strategy has pledged to spend over 20 million on pioneering eye research over the next five years. If you're personally affected by OcMMP and are interested in fundraising to help us fund cutting-edge research in this area find out how to set up a family fund.