Introduction
The word cicatricial refers to 'scarring.' Ocular cicatricial pemphigoid (OCP) is a rare autoimmune condition that affects the scarring of the conjunctiva of the eye. Conjunctiva is the inner lining of the eye. It is also known as mucous pemphigoid disorder. The disease is regarded as an autoimmune condition, which means that the body's immune system unintentionally causes inflammation and blistering on otherwise healthy tissue. A few patients have skin involvement, typically found around the head and neck or at regions of skin trauma.
Some patients have skin involvement, while others only have eye involvement. The larynx, genitalia, rectum, and esophagus are other mucosal areas that could be affected. It can be challenging to distinguish from another blistering autoimmune disease that may affect the mucous membranes, such as bullous pemphigoid. It typically manifests in late adulthood in the 50s or 60s, affects more women than men, and has a poor prognosis.
What Causes Ocular Cicatricial Pemphigoid?
It is an autoimmune disorder in which the body creates substances called antibodies that cause it to get harmed. The exact cause of the disease is unknown. The probability of acquiring cicatricial pemphigoid appears to be influenced by environmental and genetic variables. Although the exact causes of this disorder are unknown, it is not related to anything the patient has done and cannot be treated or made better by altering food or lifestyle. Numerous other autoimmune disorders exist, such as thyroid dysfunction and rheumatoid arthritis.
What Are the Signs and Symptoms of Ocular Cicatricial Pemphigoid?
The sign and symptoms of ocular cicatricial pemphigoid include:
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Dry eyes.
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Redness of the eye.
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Itching.
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Conjunctivitis (inflammation of the conjunctiva).
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Inflammation and ulcers in the lens, cornea, lobe, iris, and eyelids.
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Mucosal discharges.
Ocular cicatricial pemphigoid can progressively worsen if not treated. The stages listed below are how doctors categorize a patient's disease severity.
Stage 1 - Mild - Symptoms include persistent red eyes and conjunctival scarring (subepithelial fibrosis)
Stage 2 - Moderate - Symptoms include Increased conjunctival scarring
Stage 3 - Severe - Abnormal adhesions (stickiness) between the conjunctiva of the eyelid and the actual eyeball (during this stage, conjunctiva and eyelid begin to fuse).
Stage 4 - Very Severe - Symptoms include increased adhesions between the eyelid and the eyeball (during this stage, the eyelid and eyeball begin to fuse).
Stage 5 - End stage - Severe corneal scarring results in loss of vision.
How Is Ocular Cicatricial Pemphigoid Diagnosed?
The diagnosis of ocular cicatricial is made by:
Physical Examination.
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Blood Test - For some patients, clinicians can assess circulating autoantibodies from a patient's serum or blood; however, this is only practicable for 20 % of patients.
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Biopsy - In a biopsy, a small sample of tissue from oral mucosa or conjunctiva can be taken. This tissue is examined in a lab to check for ocular cicatricial pemphigoid.
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Staining - Using immunofluorescent staining, medical professionals can check for particular proteins in ocular cicatricial pemphigoid.
How Is Ocular Cicatricial Pemphigoid Treated?
The treatment aims to prevent the progression of Ocular cicatricial pemphigoid to an advanced stage of the disease. The cornea may suffer severe damage in the last stages of ocular cicatricial pemphigoid, which could lead to eyesight loss. Immunomodulators, which aid in regulating or normalizing the immune system, are typically used long-term.
For mild ocular cicatricial pemphigoid, the doctor will advise lubricating eye drops and placing silicone plugs inside the eye's tear drainage system are methods to treat dry eyes.
1. Medications
Depending on the severity of the disease, the doctor may prescribe some medicines, including:
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Dapsone - Can help with mucosal and eye disease.
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Triamcinolone - For minor buccal and gingival problems.
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Dapsone - Can aid with mucosal and ocular disorders.
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Triamcinolone - For mild buccal and gingival disease, Triamcinolone is applied topically.
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Azathioprine - It may be required to prevent the usage of steroids.
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Other Medications - Cyclophosphamide, Ciclosporin, Sulfasalazine, Etanercept, and Mycophenolate are also used.
2. Surgical Treatment
For many patients, surgery is the final resort option. During the advanced stages of the disease, the doctor might advise surgery to fix eyelid abnormalities. Additionally, surgery can stop the scarring of the cornea. Surgical treatment is also necessary, particularly for airway obstruction (tracheostomy) and esophagus dilations.
What Are the Complications of Ocular Cicatricial Pemphigoid?
Ocular cicatricial pemphigoid can affect the eyes in several ways if it is not treated; symptoms may worsen over time. The complications of ocular cicatricial pemphigoid include:
1. Ocular Pain - Ocular pain is caused by corneal damage
2. Insufficient Tear Production (Tear Deficiency) - Leads to severe dry eyes
3. Entropion (Trichiasis) - The condition develops when the eyelids move inward, resulting in the eyelashes touching the front of the eye resulting in scarring, infection, and irritation.
4. Others - Blindness, esophageal strictures, and airway obstructions.
Conclusion
Ocular cicatricial pemphigoid (OCP) is a rare autoimmune condition affecting the eyes' conjunctiva and skin. It is a recognized autoimmune disease defined by the generation of autoantibodies, even though the precise causes of this condition are unknown. The larynx, genitalia, rectum, and esophagus are other mucosal areas that could be affected. It can be challenging to distinguish from another blistering autoimmune disease that may affect the mucous membranes, such as bullous pemphigoid. The goal of the treatment is to reduce the growth of the condition and avoid consequences. Immunomodulators, which aid in regulating or normalizing the immune system, are typically used long-term. There is no known treatment for the disease, but with proper long-term care and close monitoring, it can manage inflammation and vision-threatening side effects.
