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Ocular Cicatricial Pemphigoid - Causes, Symptoms, and Treatment

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Ocular cicatricial pemphigoid is a rare, chronic autoimmune disease that causes blisters and scars in the eye's mucous membrane. Read about the topic in detail.

Medically reviewed by

Dr. Filza Hafeez

Published At April 3, 2023
Reviewed AtApril 1, 2024


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:

  • Dry eyes.

  • Redness of the eye.

  • Itching.

  • Conjunctivitis (inflammation of the conjunctiva).

  • Inflammation and ulcers in the lens, cornea, lobe, iris, and eyelids.

  • 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.

  • 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.

  • 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.

  • 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:

  • Dapsone - Can help with mucosal and eye disease.

  • Triamcinolone - For minor buccal and gingival problems.

  • Dapsone - Can aid with mucosal and ocular disorders.

  • Triamcinolone - For mild buccal and gingival disease, Triamcinolone is applied topically.

  • Azathioprine - It may be required to prevent the usage of steroids.

  • 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.


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.

Frequently Asked Questions


What Other Term Is Used to Refer to Ocular Cicatricial Pemphigoid?

Ocular cicatricial pemphigoid (OCP) is also known as Mucous Membrane Pemphigoid (MMP). These terms can be used interchangeably. OCP, characterized by autoimmune conjunctivitis, results in the scarring (cicatrization) of the conjunctiva. Without proper treatment, OCP has the potential to progress to blindness.


Where Is the Most Common Location for Ocular Cicatricial Pemphigoid in the Oral Cavity?

In the oral cavity, ocular cicatricial pemphigoid commonly affects the gums, making them one of the primary sites of involvement. This autoimmune condition leads to inflammation and scarring of the mucous membranes, including those in the mouth. The gums are particularly susceptible, often causing discomfort and oral health complications for individuals affected by this condition. Treatment typically focuses on managing symptoms and reducing inflammation.


What Are the Available Treatments for Ocular Cicatricial Pemphigoid?

Available treatments for ocular cicatricial pemphigoid include a combination of medications and therapies to reduce inflammation, manage symptoms, and prevent complications. Common approaches involve immunosuppressive drugs, such as corticosteroids, other immune-modulating medications, and topical treatments to alleviate discomfort and promote healing in affected areas. Regular monitoring and supportive care are also essential to the management plan to ensure patients' best possible quality of life.


Which Drug Is Typically Considered the Primary Choice for Treating Ocular Cicatricial Pemphigoid?

The primary choice for treating ocular cicatricial pemphigoid is often Dapsone, a medication that helps suppress the autoimmune response and reduce inflammation. Dapsone is frequently used alongside other immunosuppressive drugs to manage symptoms effectively. However, the specific treatment plan may vary based on the severity of the condition and the individual's overall health, requiring close consultation with a healthcare professional for personalized and optimal management.


Can Pemphigoid Lead to Blindness?

Yes, pemphigoid can lead to blindness, especially in ocular cicatricial pemphigoid cases where the inflammation and scarring affect the eyes. If left untreated or not properly managed, the condition can cause significant damage to the ocular tissues, leading to vision impairment or, in severe cases, blindness. It underscores the importance of early diagnosis and appropriate medical intervention to preserve vision and prevent irreversible damage.


Is There a Cure for Pemphigoid?

Bullous pemphigoid can naturally resolve within a few months to up to five years. Treatment aims to heal blisters and relieve itching, commonly involving corticosteroid medications like Prednisone and immune-suppressing drugs to manage the condition effectively.


How Severe Is Pemphigoid?

The severity of pemphigoid can vary widely among individuals. While some people experience mild symptoms that can be managed effectively with medications, others may face more severe complications. It is crucial for individuals diagnosed with pemphigoid to have regular medical follow-ups and adhere to prescribed treatments to prevent worsening and maintain overall well-being.


Does Ocular Cicatricial Pemphigoid Cause Pain?

Yes, ocular cicatricial pemphigoid can be painful. The inflammation and scarring in the eye tissues can cause discomfort, irritation, and pain, impacting the overall quality of life for individuals affected by this condition. Proper medical management and pain relief measures are essential to alleviate these symptoms and enhance the patient's comfort.


What Sets Pemphigus and Pemphigoid Apart?

The main difference between pemphigus and pemphigoid lies in the specific skin layers they affect. Pemphigus involves the uppermost layer of the skin (the epidermis), forming fragile blisters that easily rupture. In contrast, pemphigoid affects the epidermis's lower layer and the dermis's upper layer, resulting in denser blisters that do not rupture as easily. Additionally, pemphigus often involves mucous membranes in the mouth and can be more severe, while pemphigoid primarily affects the skin and mucous membranes, typically causing less severe symptoms.


Which Vitamin Deficiency Is Associated with Pemphigoid?

Pemphigoid is associated with a deficiency in vitamin B12. Adequate levels of this vitamin are essential for overall skin health, and a deficiency can contribute to developing or exacerbating pemphigoid symptoms. Therefore, individuals with pemphigoid need to maintain a balanced diet and, if necessary, take vitamin B12 supplements under medical supervision to support their overall health and potentially alleviate symptoms related to the condition.


What Is the Typical Duration of Pemphigoid?

The duration of pemphigoid can vary widely from person to person. Some individuals may experience acute episodes that resolve within a few months with appropriate treatment, while others may have a chronic condition that persists for several years. Continuous medical management is essential to control symptoms, promote healing, and prevent flare-ups. Regular follow-ups with healthcare professionals are crucial to monitor the condition's progress and adjust the treatment plan.


Can Ocular Cicatricial Pemphigoid Impact the Brain?

Yes, although it is rare, ocular cicatricial pemphigoid can impact the brain. The inflammation associated with this condition can sometimes extend to the central nervous system, leading to neurological symptoms. However, this complication is uncommon and requires careful evaluation and management by healthcare professionals. Patients with ocular cicatricial pemphigoid should be vigilant about any unusual neurological symptoms and promptly report them to their healthcare providers for proper assessment and intervention.


Is Ocular Cicatricial Pemphigoid Inherited Genetically?

Ocular cicatricial pemphigoid is not primarily considered a genetic disorder. While there is a genetic component to autoimmune conditions in general, the exact cause of ocular cicatricial pemphigoid is not fully understood and is likely multifactorial, involving a combination of genetic predisposition and environmental factors. Individuals with a family history of autoimmune disorders need to inform their healthcare providers, as genetics can sometimes play a role in autoimmune conditions.


Is Ocular Cicatricial Pemphigoid a Cancerous Condition?

No, ocular cicatricial pemphigoid is not inherently cancerous. It is an autoimmune disorder that affects mucous membranes, including those in the eyes, mouth, and other body areas. However, if left untreated or if complications arise, such as chronic inflammation or infections, there can be an increased risk of developing squamous cell carcinoma, a type of skin cancer. Regular monitoring, early detection, and appropriate medical management are crucial to prevent complications and maintain the patient's health.


What Is the Mortality Rate Associated with Pemphigoid?

The mortality rate associated with pemphigoid is generally low, especially with advancements in medical treatments. With proper management and timely medical intervention, most individuals with pemphigoid can lead a relatively normal life expectancy. However, the mortality rate can vary based on the severity of the condition, the effectiveness of treatments, and any complications that may arise.

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Dr. Filza Hafeez



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