A rare, persistent, blistering, and scarring condition that impacts both the oral and ocular mucosa is called ocular cicatricial pemphigoid. The esophagus, genitalia, rectum, larynx, and other mucosal areas could also be impacted. The most feared side effect, which might result in blindness, is scarring of the damaged eye’s mucosa. A type of mucous membrane pemphigoid (a rare group of chronic autoimmune diseases) that affects the eyes is called ocular cicatricial pemphigoid (OCP).
Ocular cicatricial pemphigoid (OCP) is an uncommon ocular surface condition typically brought on by an immunological response of the patient’s body to conjunctival and ultimately corneal cells. Dryness and irritability are often the first signs. The symptoms may increase over months or years, and vision may be impacted. The diagnosis is typically made during a routine office visit, and a small conjunctival biopsy may occasionally be used to confirm it. Eye lubrication and oral anti-inflammatory drugs are typically used to treat OCP. OCP is frequently treatable; however, some individuals continue to deteriorate despite vigorous treatment.
The fragile mucous membrane known as the conjunctiva lines the inside of the eyelids and encircles the eyeball. Conjunctivitis (red, swollen conjunctiva) and scarring brought on by these autoantibodies frequently lead the eyelashes to bend inward and scrape the eye, making the condition worse. Cicatricial refers to scarring, and pemphigoid was the condition’s original name. Usually, one eye is damaged more than the other, but both eyes are typically affected. A minority of patients experience rapid illness progression over a matter of weeks, while the majority experience delayed disease progression over the course of months or years. In serious cases, early diagnosis improves results. With effective treatment, the disease’s consequences may typically be managed and both comfort and vision preserved.
It is unclear to us what causes ocular cicatricial pemphigoid. Since it is known that the disease has a genetic component, it isn’t a hereditary condition, and its initial cause is unknown. Similar to other autoimmune illnesses, components of the body are wrongly attacked by the immune system. When you have MMP, your body’s immune system produces autoantibodies that attack the layer directly beneath the skin or mucous membranes, resulting in swelling and scarring. Although it rarely happens in the eyes, blistering can happen elsewhere. Ocular Cicatricial Pemphigoid is not hereditary, it cannot be “caught” by another person, and it is not brought on by certain foods or allergies. Men and women over the age of 40 are typically affected (but younger adults and, very occasionally, children.
Signs and Symptoms:
Conjunctivitis, which manifests in the eyes as red, painful, and sticky eyes, can be moderate and intermittent or serious and ongoing as the condition progresses. These symptoms can cause a lot of discomfort and irritation and do not get better when treated with antibiotics or eye drops. Even when conjunctival scarring develops promptly, it can be challenging to observe with the unaided eye until the condition is pretty well established.
The conjunctival fornix, which is the pit between the eyelids and the eyeball, can become less deep as the scarring and inflammation worsen, which may manifest as a droopy lid or a narrower separation between the lids. The eyelashes may start to scratch on the outer layer of your eye and the eyelids could cave in. Trichiasis is the term for this. Scarring and inflammation can also disrupt the tear-producing glands, which results in dry eyes.
A tiny sample of tissue, known as a biopsy, is obtained from the afflicted mucous membrane and/or the epidermis under local anesthesia, and tests are then performed to confirm the diagnosis of MMP. A straight immunofluorescence test is what is known as this test. As part of the serology testing, the blood is also examined for the existence of autoantibodies. However, in around 25% of instances, the eyes become the only or initial afflicted region, and an ophthalmologist makes the diagnosis of OMMP. The same tests are then performed as previously stated, with the exception that conjunctiva biopsies from both eyes’ conjunctivas are collected using anesthetic eye drops, as well as typically one other place (often the mouth or skin).
The condition can still be identified as ocular cicatricial pemphigoid even if these tests come back negative by ruling out additional causes of conjunctival inflammation and scarring. An expert in ocular cicatricial pemphigoid will collaborate with the nearby ophthalmology department to validate the diagnosis, provide shared care, and enhance your therapy in this case. Their viewpoint can be very beneficial.
Treatment for the Disease:
The immunosuppressive/immunomodulatory drug that dermatologists and we have found to be effective in treating patients with growing ocular cicatricial pemphigoid is not a cancer chemotherapy drug that causes constant sickness, hair loss, and the possibility of a lethal reaction to the drug itself. Pharmaceuticals are not only extremely successful when used correctly by a person who is, by virtue of both education and practical expertise, an expert in how to make use of such pharmaceuticals, but they are also often relatively safe. On the latter point, we have written and demonstrated that using such drugs in our hands is actually vastly safer than using systemic cortisone-type drugs over an extended period of time.
Patients with a range of autoimmune disorders, such as ocular cicatricial pemphigoid, have a bright future ahead of them. The “susceptibility gene” for this condition has been found, and the target antigen that the patient’s white blood cells assault when they develop ocular cicatricial pemphigoid has also been located.