Ocular cicatricial pemphigoid (OCP) is a rare autoimmune disease. Without proper treatment, it can damage the eyes and cause vision loss.

OCP is a type of mucous membrane pemphigoid (MMP). MMP is a rare autoimmune disease that causes blistering lesions on mucous membranes of the body.

OCP affects the eyes and causes chronic conjunctivitis (pink eye). It may also affect other mucous membranes and the skin. OCP affects around 60% to 70%Trusted Source of people who have MMP. Around 1 in 8,000 to 1 in 46,000 people with eye health conditions may have OCP.

A woman applying eye drops in the mirror-1Share on Pinterest
Westend61/Getty Images

OCP is a chronic autoimmune disease that primarily affects the surface of the eyes. It is a subcategory of MMP and can lead toTrusted Source permanent vision loss if a person does not receive treatment.

The disease causes chronic and relapsing conjunctivitis in both eyes. OCP occurs as a result of inflammation of the conjunctiva — the clear layer over the white of the eye and inside the eyelid — and leads to cicatrizing conjunctivitis, which causes scarring of the eye.

Key features of OCP include:

  • chronic conjunctivitis (pink eye)
  • severe dry eye syndrome
  • scarring of the eye tissues
  • an inward-turning eyelid
  • eyelashes that grow toward the eye
  • potential vision loss

In the early stagesTrusted Source, OCP can have nonspecific symptoms, which can lead to misdiagnosis or delayed treatment. The symptoms can be similar to those of dry eye, such as:

Unlike eye infections, OCP causes little to no discharge from the eye.

In the later stages of the disease, additional symptoms may develop, including:

  • clouding of the eye
  • fusion of the upper eyelid with the lower eyelid
  • symblepharon (an abnormal joining of the two layers of the conjunctiva)

OCP is twice as common in females as in males and usually begins around 60 years or age or later.

While OCP primarily affects the eyes, it can also affect other mucous membranes throughout the body.

Aside from the eyes, OCP most commonly affects the mucous membranes of the mouth and can cause red, sore gums.

It can also affect mucous membranes in the esophagus, causing difficulty swallowing, reflux disease, weight loss, and life threatening aspiration. Additionally, it can affect membranes in the throat, leading to narrowing of the airways, hoarseness, cough, voice disorders, and changes in breathing.

Less commonly, OCP can affect the mucous membranes in the following areas:

  • skin
  • nose
  • genitals
  • anus

OCP is an autoimmune disease in which the body’s immune system mistakenly attacks the surface tissues of the eyes. Experts do not fully understand the exact trigger for the disease.

In most cases, there are no known risk factors for OCP. Research suggests that a combination of genetic and environmental factors may cause the body to produce autoantibodies.

Autoantibodies are substances the immune system produces that attack the body’s own tissues. The production of autoantibodies may trigger an inflammatory response, which may cause OCP to develop.

Theories suggest that part of an antigen (a foreign substance in the body that provokes an immune reaction) may spread in severe conjunctivitis and cause OCP. This may be the case when OCP occurs after Stevens-Johnson syndrome or toxic epidermal necrolysis or after the use of glaucoma eye drops.

OCP may have links to other autoimmune conditions, including:

Doctors diagnose OCP based on clinical signs of the disease and biopsy results. To confirm clinical findings of OCP, a doctor will take a biopsy of the conjunctiva tissue or other affected tissues in the body.

The biopsy can detect specific immunoglobulins, or antibodies, in a part of the conjunctiva tissue called the conjunctival basement membrane zone (CMZ). The presence of the following antibodies in the CMZ indicates OCP:

  • IgG
  • IgA
  • IgM
  • C3

After an OCP diagnosis, doctors use staging systems to evaluate OCP progression and monitor the disease. Although OCP affects both eyes, the signs and progression of the disease may be different in each eye, so doctors grade each eye separately.

Treatment for OCP aims toTrusted Source control inflammation and prevent disease progression. Treatment may involve:

  • undergoing immunomodulatory therapy
  • managing dry eye symptoms
  • preventing corneal damage
  • using scleral contact lenses

Topical and local treatments for OCP

Treating dry eye symptoms is important to help prevent the corneal epithelium (the covering of the cornea) from breaking down. Treatment for these symptoms may include:

Increasing the volume of tears on the eye’s surface may help promote tissue healing. Possible methods include:

  • silicone punctal plugs (small devices that fit into the tear ducts of the eyes)
  • scleral lenses (rigid contact lenses that help protect against dryness and irritation)

Eyelid care to treat blepharitis is important to help prevent OCP symptoms from worsening. Treatment for blepharitis may include:

In some cases, people may need surgery to correct symptoms or repair damage.

Systemic treatments

Systemic treatment, which targets the whole body, is the only treatment that will preventTrusted Source OCP progression. Dapsone, an antibiotic, is usually the first-line treatment for mild to moderate OCP.

In severe cases or if OCP does not respond to first-line treatments, treatment may involve:

  • corticosteroids with immunosuppressant therapy
  • biologics with intravenous (IV) immunoglobulin therapy

If there is a high suspicion of OCP, even if a biopsy does not indicate OCP, immunosuppression treatment may still be necessary.

Some treatments may have side effects. It is important for a person to discuss any potential side effects or treatment risks with a doctor.

Eye health resources

Visit our dedicated hub for more research-backed information and in-depth resources on eye health.

Without treatment or in severe cases in which OCP does not respond to treatment, the disease may lead to permanent vision loss or blindness. Advanced OCP is more likely to progress than mild OCP.

In around 90%Trusted Source of cases, systemic treatment can stop OCP from progressing. OCP may recur in 20% to 30% of cases. Even if OCP is in remission, a person will need to manage it throughout their life.

Is there a cure for OCP?

There is currently no cure for OCP. Treatments aim toTrusted Source control inflammation, manage symptoms, and prevent disease progression.

Can OCP lead to blindness?

Without treatment or in aggressive cases that do not respond to treatment, OCP may lead to severe vision loss or blindness in the later stages of the disease.

How often should someone with OCP see their eye doctor?

Regular follow-ups are essentialTrusted Source for managing OCP. An ophthalmologist will determine the frequency of checkups based on the severity of each individual case.

OCP is an autoimmune disease that mainly affects the eyes. Without treatment, it can lead to permanent vision loss.

Treatment can help manage symptoms and prevent disease progression. OCP requires long-term management and frequent doctor’s visits.