What is ocular cicatricial pemphigoid?
Ocular cicatricial pemphigoid or OCP is a chronic rare idiopathic autoimmune disease that targets the mucous membranes of the eyes and skin. An autoimmune disease is characterized by an overactive immune system that incorrectly targets its own natural cells, promoting inflammation in the body within healthy tissues. Because OCP is idiopathic, there is nothing that one can do to prevent the condition as it is not caused by anything the patient has done. This condition happens most commonly in older or middle aged patients and occurs more commonly in women than men. It is seen bilaterally and is progressive in nature. It results in scarring of the cornea and conjunctiva. Diagnosis of the condition is based on examination and subsequent clinical signs. A biopsy of the area can be done and sent for analysis. The use of colored dyes can help illuminate scarring of the eyes. The scarring disrupts the smooth exterior of the eye and can cause damage to the eye as well as vision changes. Unfortunately the condition is chronic and can relapse and reoccur. Therefore, it is imperative to work with your eye doctor to improve symptoms and prevent deterioration of vision.
How does OCP affect the eyes?
OCP causes inflammation within the tissues, most commonly in the conjunctiva. The conjunctiva is a very thin outer connective tissue that covers the white part of your eye. The inflammation can cause extreme dryness and discomfort. If left untreated, OCP can lead to scarring of the front surface of the eye—the cornea. This causes vision loss and changes, the worst end result being blindness.
Risk factors for ocular pemphigoid
OCP is idiopathic in nature and is therefore not caused by anything the patient has done. However, advancing age can be a risk factor due to the tendency of the condition to occur in older individuals. Females are also more likely to be diagnosed with OCP. However, these are both risk factors that are out of our control. Regular eye exams are needed in order to properly monitor OCP and preserve optimal vision.
Symptoms of OCP
- Red or dry eyes
- Excessive tearing
- Itchy eyes
- Skin changes including lesions or scars
- Inward turning of eyelashes
- Corneal defects
- Corneal infections
- Corneal neovascularization
- Symblepharon—adhesion between the eyelid and front surface of the eye
Complications of ocular pemphigoid
If OCP is left untreated, it can lead to a myriad of complications. Severe dry eyes is one of the most common complications. This can be uncomfortable for the patient and negatively affects one’s vision. If eyelashes that are turned inwards are left in place, it can lead to scarring, infection, or ulceration. Eye pain is common when damage to the cornea is involved. Glaucoma is a possible complication of OCP but is not as easily treated as normal variants of glaucoma.
Treatment and management
Your eye doctor will be able to help you initiate proper treatment for ocular cicatricial pemphigoid. There is no cure for pemphigoid, but it is possible to carefully control inflammatory processes and therefore prevent negative side effects to one’s vision. Inflammation is the mechanism of action of OCP and is therefore inflammatory processes are the mainstay target of treatment. Treatment is important to help suppress progression of the condition and prevent blindness. Surgeries should be avoided as long as possible to avoid unnecessary trauma to the eye. Trauma can exacerbate the disease and speed up progression. Some examples of treatment options are:
- Systemic medications
- Immunomodulatory drugs could be used to help suppress the immune system and limit the body’s attack on itself. This helps reduce the body’s reaction enough to prevent scarring of the cornea which would lead to blindness.
- Artificial tears
- Preservative free artificial tears could be utilized to improve the lubrication of the eyes and therefore reduce unwanted friction that could further damage to the eye. Artificial tears could also be used to help improve patient comfort as many OCP patients complain of overly dry eyes.
- Steroid eye drops
- Steroids work to suppress inflammation and therefore work well to decrease the inflammatory processes seen in OCP. This can help reduce the amount of scarring and damage that occurs as well.
- Epilation of lashes
- Epilation involves the removal of lashes that are turned inward. This is important to prevent irritation of the eyes and further scarring.
- Amniotic membranes
- Amniotic membranes can be placed on the eye and help promote healing. These membranes have anti-inflammatory properties and therefore are beneficial in this condition. It can help provide a scaffold to promote regrowth of damaged corneal epithelial cells.
- Scleral contact lenses
- Scleral contact lenses are larger than typical soft contact lenses and rest on the whites of the eyes rather than the cornea. This provides room to hold a fluid reservoir beneath the contact and above the cornea. The fluid reservoir lubricates the eye, providing a soothing effect for the patient and helps treat dry eyes. The large contact can also prevent further mechanical damage from the lids while blinking.
- Bandage contact lenses
- Bandage contact lenses can help improve the sensation of a foreign object in the eye. This can help in cases of inward turned eyelashes and prevents scarring.
- Artificial corneal transplant
- In extreme cases of scarring, an artificial corneal transplant may be needed in order to improve the patient’s vision.
- Lid hygiene
- Proper lid hygiene can help improve symptoms of blepharoconjunctiviits and dry eye.
- Warm compresses
- Daily warm compresses applied to closed eyes for a duration of 5 minutes can improve dry eye symptoms by allowing for better meibomian gland secretions.
- Punctal plugs
- Small plugs can be placed within the openings of your drainage system (the puncta) in order to prevent tear outflow. This will increase surface level tears and help provide lubrication to the eye.
FREQUENTLY ASKED QUESTIONS ABOUT OCP
How are autoimmune diseases like OCP diagnosed in the eye?
Your eye doctor will evaluate the eye and go through a thorough medical history with you. Your doctor will evaluate your vision, use vital dyes to stain the eye to look for scarring or defects in the cornea, take a look at your eyes tear production, and evaluate for dry eye. Clinical signs are imperative in diagnosis and is confirmed via biopsy.
What are some differential diagnoses for OCP? What else could it be?
OCP can present similarly to a couple other conditions and therefore requires careful observation and a knowing eye. Some diseases that may have similar manifestations in the eye are: chemical burns, graft vs host disease, sjogrens syndrome, stevens-johnson syndrome, and sarcoid to name a few.
Can OCP affect other parts of the body?
In addition to ocular manifestations, patients may develop skin blistering or scarring. This can occur in the mouth, genitals, or even internally in the intestinal tract. The inflammation seen in OCP can occur anywhere in the body where there is healthy tissue, most commonly in mucosal linings.
What is the benefit of diagnosing pemphigoid?
OCP is a progressive disease and can ultimately lead to blindness. Early diagnosis is important in order to initiate treatment that can help preserve vision. It is important to improve quality of life for patient as well as they can experience extreme discomfort in cases of dry eye or pain from scarring.