Overview

Ocular Cicatricial Pemphigoid & Immune-Mediated Oculocutaneous Diseases

Ocular Cicatricial Pemphigoid & Immune-Mediated Oculocutaneous Diseases

Ocular cicatricial pemphigoid, also known as mucous membrane pemphigoid (MMP), is an autoimmune disease that can cause scarring in the conjunctiva of your eyes. The conjunctiva is the inside lining of your eyes that keeps a smooth surface when you blink or close your eyes.

When there is scarring in your conjunctiva, your eye can become damaged, causing vision loss.

MMP can also cause scarring in other mucous membranes, including your mouth and throat. This disease usually affects people over 60, and more women have MMP than men.

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Symptoms of Ocular Cicatricial Pemphigoid

As an autoimmune disease, MMP uses your immune system to cause damage to your body in several areas. Symptoms may include the following:

  • eye redness
  • mucous discharge in your eyes
  • ulcers in your conjunctiva, mouth, genitals, and anus
  • hoarseness and difficulty swallowing

Stages of MMP

Without treatment, MMP can become worse over time. Doctors use these stages below to classify how severe a patient's MMP is:

  1. Stage I—Mild: Chronic red eyes and early scarring on the conjunctiva (subepithelial fibrosis) 
  2. Stage II—Moderate: Increased scarring of the conjunctiva, which causes the fornices inside the conjunctiva to become shorter
  3. Stage III—Severe: Abnormal adhesions (stickiness) between the conjunctiva of the eyelid and the eyeball itself (during this stage, your conjunctiva and eyelid begin to fuse together)
  4. Stage IV—Severe: Increased adhesions between eyelid and eyeball (during this stage, your eyelid and eyeball begin to fuse together)
  5. End stage—Irreversible Vision Loss: Severe scarring in the cornea causes vision loss

Problems & Complications

If left untreated, MMP can cause many problems in your eyes (also called complications). Over time, symptoms can become worse. Problems can include the following: 

  • chronic tear deficiency causing severe dry eyes (your eyes don't produce enough tears)
  • entropion/trichiasis—this happens when your eyelids turn inwards, causing your eyelashes to touch the front surface of your eye. This in turn causes irritation, infection, and scarring
  • eye pain from damage to the cornea
  • loss of vision in the worst cases

How is MMP Diagnosed?

  • Biopsy: doctors can collect a small amount of tissue in your conjunctiva or oral mucosa, then examine this tissue in a lab to check for signs of MMP.
  • Staining: doctors can also use immunofluorescent staining to look for specific proteins found in MMP, such as complement 3.
  • Blood test: for some patients, doctors can measure circulating autoantibodies from a patient's serum or blood, but this is only possible for 20 percent of patients.

 

Management & Treatment

The goal of treatment is to stop MMP from progressing to end-stage disease. The end stages of MMP can cause extensive damage to the cornea, which may ultimately cause vision loss.

  • For mild MMP, your doctor will recommend lubricating eye drops to treat the dry eye that MMP causes.
  • Another treatment for mild MMP is to place silicon plugs into the tear drainage system in your eye. These plugs keep your own tears in your eye longer and make your eyes moister.

Medications

If your MMP is more severe, your doctor may prescribe the medication dapsone.

Stronger medications include azathioprine and mycophenolate mofetil. These medications will decrease your body's immune response.

If your MMP is still progressing (getting worse), other medications can be used for treatment, including IV immunoglobulin or rituximab.

Surgery Options

We understand that surgery is a last resort for many patients. However, in some cases, surgery may be the best option. Your doctor may recommend surgery to correct eyelid deformities during the late stages of MMP. Surgery can also prevent your cornea from scarring.

If your cornea already has scarring, your doctor can help you decide if a corneal transplant would be a good option.

Multidisciplinary & Personalized Care

Since MMP can become worse quickly, patients with MMP need close follow-up from a team of experts.

Our multidisciplinary team includes ophthalmologists specializing in cornea and eyelids (oculoplastics) as well as dermatologists who are experts in diagnosing and treating MMP with strong immunosuppressive medications (medications that weaken your immune system's response).

Our team works together to ensure patients get the best care and latest treatment to prevent potentially devastating complications, including vision loss.