Ocular Cicatricial Pemphigoid

What is Ocular Cicatricial Pemphigoid (OCP)?

Ocular cicatricial pemphigoid (OCP) is the eye disease affecting patients with mucous membrane pemphigoid (MMP). The most up-to-date term for the condition is ocular mucous membrane pemphigoid (ocular MMP).

OCP is a rare, potentially sight-impairing autoimmune disease affecting the conjunctiva. The conjunctiva is the transparent mucous membrane lining which covers the white of the eye and the undersurface of the eyelids. In OCP the conjunctiva becomes red, inflamed and progressively scarred, leading to inturned eyelashes.

“Ocular” means eye, “cicatricial” means scarring, and “pemphigoid” is an autoimmune blistering disease.

OCP can be isolated to the eye alone (in 18 to 50% of patients with eye disease). More commonly it occurs as part of the whole-body condition called mucous membrane pemphigoid (MMP), which causes painful blistering, inflammation & scarring of the moist surfaces of the body (known as mucous membranes) including the mouth and gums, the lining of the nose, oesophagus (the swallowing tract), larynx (voice box), genitals, rectum, ear, eyes and skin. About 70% of whole-body MMP patients have eye involvement.

The following information is provided as a guide only. Careful assessment and treatment by an eye specialist (ophthalmologist) who has the expert training and experience to accurately determine your diagnosis and best treatment approach is important.

What causes Ocular Cicatricial Pemphigoid (OCP)?
OCP is an autoimmune disease where the body’s immune system is overactive and mistakenly attacks otherwise healthy tissue, causing blistering, inflammation and scarring. Examples of other autoimmune diseases include rheumatoid arthritis and lupus.

Ocular cicatricial pemphigoid can be triggered by drugs or eye drops, or occasionally occurs following other diseases such as Stevens Johnson Syndrome, but in the majority of patients the trigger is not identifiable. The overactive immune system produces antibodies which mistakenly recognize parts of the body as foreign, leading to blistering damage, inflammation and scarring.

OCP is not contagious and not affected by diet or lifestyle.

Who is usually susceptible to OCP?
OCP predominantly affects adults aged 40 – 70, with women affected slightly more often than men. However it can occur in 20-30 year olds, and also in children.
How is OCP diagnosed?
A small sample of tissue (biopsy) is taken from the eyes and usually also the mouth. These biopsies are evaluated under a fluorescence microscope and tested for antibodies associated with MMP. Whilst biopsies from skin and mouth MMP lesions are less than 10% likely to give false negative results, unfortunately biopsies from the eye can give a false negative result in 30-50% of cases. So a positive result from the eye confirms the diagnosis, but a negative result from the eye does not necessarily mean that the diagnosis is not MMP. In a proportion of patients with negative biopsy results, treatment with immunosuppressive therapy (which suppresses an overactive immune system) in a similar manner to patients with positive biopsy results, may be necessary.

A blood test may also be used to detect the antibodies in the circulation, but they are detectable in less than 30% of ocular MMP patients.

If it is likely that you will require immunosuppressive medication (which suppresses an overactive immune system), Ms Saw will request that you have baseline pre-treatment blood tests (including a full blood count, electrolytes & renal function, liver function tests) to establish if there are any pre-treatment abnormalities which require investigation or treatment, or which may alter her decision regarding which immunosuppressive medication would be best for your situation. These blood tests are usually done at the time that the biopsies are taken.

Once a diagnosis of OCP is made, you may be referred to a Dermatologist, an Oral Medicine specialist and/or an Ear, Nose & Throat (ENT) specialist, depending on your symptoms. Multi-disciplinary care is essential for successful management of OCP/ MMP.

What can I do?
  • Hot eyelid compresses and massage to express “oily tears” to the surface of your eyes once every day, as part of your daily hygiene routine, will make your eyelids and eyes as healthy as possible.
  • Keep your eyes moist with lubricating drops, gel or ointment – avoid letting dryness cause further damage to the eye.
  • Use your eye drops and take the immunosuppressive medication as instructed by your specialist
  • If any medication makes you feel very unwell, stop it and contact your OCP specialist.
  • If eyelashes are rubbing your eyes, either have them removed by a local optometrist or A&E unit, or enlist the help of a relative or friend using tweezers & magnifying glass. Trying to do it yourself can be difficult. Eyelashes rubbing the eyes can cause an ulcer on the cornea that then gets infected and leads to irreversible sight loss.
  • Stop smoking and reduce alcohol intake to recommended limits
Will the immunosuppressive treatment make me feel unwell?

All medications potentially have side effects. If the Ocular Cicatricial Pemphigoid disease itself is making you unwell, immunosuppressive medication is prescribed to relieve this. Most people tolerate immunosuppressive medication well. If you are having side effects from the medication then the dose of the medication could be reduced, or switched to a different medication, in order to find the medication which controls your OCP with minimum side effects.

Can I go blind?

Unfortunately some patients can permanently lose their eyesight due to OCP causing permanent scarring of the cornea, the clear front window of the eye, as a consequence of severe dryness, corneal infection or damage to limbal stem cells.

What are the symptoms of Ocular Cicatricial Pemphigoid (OCP)?

The commonest symptoms of OCP are:

  • Inturned eyelashes (trichiasis) rubbing the eye ***
  • Red, gritty sore or dry eyes with sensitivity to light
  • Conjunctivitis (red sticky eye) that keeps returning despite courses of antibiotic eyedrops
  • Droopiness and closing of the eyelid

*** If you have inturned eyelashes that often need to be pulled out, it is essential to have a careful assessment by an eye consultant who is expert in diagnosing ocular cicatricial pemphigoid, to evaluate the underlying cause of the inturned eyelashes. ***

How will I know if OCP is affecting other parts of my body?

Symptoms of mucous membrane pemphigoid (MMP) affecting other parts of the body including mouth ulcers, bleeding gums, nose bleeds, skin rash or blisters, difficulty swallowing, a hoarse voice, blood in the motions, genital sores and difficulty hearing. It is important to let your OCP specialist know if you are experiencing any of these symptoms.

How is OCP treated?

Because it is an autoimmune condition, treatment of OCP requires strong oral medication as tablets or injections, called immunosuppressive therapy (which suppresses the overactive immune system). This treatment may be required for several months, years or indefinitely. Regular blood tests are required to check for unwanted effects of treatment in the first weeks to months of treatment. Immunosuppressive medication used in OCP include mycophenolate, cyclophosphamide, methotrexate, and dapsone. Limited courses of oral prednisolone (steroid) may also be used. 

Eye drops cannot alter the progressive nature of the condition – only immunosuppressive medication can do this – but steroid drops may be used for short periods to reduce inflammation. Long term use of steroid eye drops can lead to eye infections, glaucoma and cataract so they are not used long term in OCP.

Dry eye is common in OCP, due to both (a) scarring and (b) Meibomian gland blockage. Lubricating eye drops/gel/ointment to maintain moisture on the eye, are essential to treat the dry eye.

Blepharitis and Meibomian gland dysfunction is common in OCP, and worsens dry eye and eye inflammation.

  • Daily hot compresses with eyelid massage are essential to express “oily tears” from blocked Meibomian glands, to treat dry eye in OCP.
  • Courses of oral Doxycycline or Lymecycline help to reduce inflammation, unblock Meibomian glands and improve tear film quality.
  • Taking omega 3 fish oil supplements has been shown in some studies to improve Meibomian gland function and dry eye.
What is the long term disease course in OCP?

In a few patients, the condition can go into remission for 1 year or more and the immunosuppressive medication can be stopped. However the majority of MMP patients continue on oral immunosuppressive medication for life.

Is there any treatment which can put the disease into remission?

The only medication which has been reported to put Ocular Cicatricial Pemphigoid into remission for more than 1 year is cyclophosphamide, which is effective but can cause side effects. 

What research is currently being carried out in OCP?

Ms Saw is currently involved in planning several research projects involving OCP:

  • A laboratory research grant investigating inflammatory dendritic cell pathways in an in vitro model.
  • A randomized clinical trial of Rituximab in treatment-recalcitrant OCP.

If you would like to receive more information about, or would like to become involved in any of the research projects we would be delighted to hear from you.

Useful links:

 

PEM Friends (for people in the UK who suffer from Pemphigus or Pemphigoid) http://pemfriends.co.uk

The International Pemphigus and Pemphigoid Foundation http://www.pemphigus.org

The British Association of Dermatologists http://www.bad.org.uk

 

Valerie Saw has treated hundreds of patients with OCP and is one of the most experienced UK experts in Ocular Cicatricial Pemphigoid.

 

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