Untreated mucous membrane pemphigoid (MMP) can lead to blindness, swallowing difficulty, and other complications. Studies suggest a potential link between MMP and cancer; therefore,  getting an early diagnosis and individually tailored treatment is crucial.  

What Is Mucous Membrane Pemphigoid?

Other names: Cicatricial pemphigoid, mucosal pemphigoid, and mucosynechial pemphigoid. 

Mucous membrane pemphigoid is a group of long-term disorders that cause blisters on your mucous membranes, occasionally the skin. 

The term “mucous membrane pemphigoid” consists of two words:

1. Mucous membrane: Also called mucosa, the mucous membranes line the insides of organs and cavities. 
2. Pemphigoid: Pemphigoid refers to tight bubbles of fluid (blisters) that do not open easily. 

The most commonly affected sites are the mouth and eyes. However, other areas may also be involved, such as your nose, throat, and genitals. 

How Common Is Mucous Membrane Pemphigoid?

MMP is a rare condition. According to the Genetic and Rare Disease Information Center (GARD), it affects fewer than 5,000 Americans [1].

Furthermore, it is more common in older adults (50 years or older), and women are more likely than men to get this disorder. 

What Causes Mucous Membrane Pemphigoid?

The exact cause is unknown. However, health experts believe a faulty immune response might be the culprit. For this reason, some consider it an autoimmune disorder. 

In individuals with this disorder, the immune system produces proteins (autoantibodies) that attack the mucous membranes of the mouth, eyes, or other sites. 

Researchers have yet to understand why this happens. However, they believe a combination of genetic and environmental factors may play a role. 

In a minority of cases, this disorder may result following certain prescription medications or surgery. 

Studies also suggest the presence of specific autoantibodies may increase the risk of cancer [2,3].

What Are the Symptoms of Mucous Membrane Pemphigoid?

Symptoms can vary depending on the site involved. 

For example, the blisters in the mouth — oral mucous membrane pemphigoid — appear as red, painful ulcerated patches on the inner cheeks, gums, or roof of the mouth. 

On the other hand, the symptoms of ocular MMP, which affects the eyes, can include:

• A feeling of roughness on the inside of the eyelids
• Red or painful eye
• In-turning eyelashes
• Mucous discharge in the eye
• Shrinking of the space between the eye and eyelid
• Sticking of the eyeball to the eyelid (partial or complete)
• Thickening of the eye’s surface
• Blindness (in severe cases)

Blisters that form on the nose, throat, and genitals may heal with scarring. Scarring in the food pipe (esophagus) can make swallowing difficult. 

If the skin is involved, blisters often appear on the scalp, face, trunk, or limbs. 

How Is Mucous Membrane Pemphigoid Diagnosed?

Diagnosis begins with a physical exam, which helps your provider determine the location and type of blisters. If they suspect MMP, they will order additional tests and procedures, such as:

• Biopsy: Your provider removes a sample of tissue from the affected site and analyzes the sample under a microscope. 
• Immunofluorescence assay: The sample is stained with a chemical, making it distinctly visible under the microscope.
• Blood test: In some cases, a blood sample can reveal the presence of autoantibodies in the blood. 

Moreover, you may need imaging studies such as CT scans if your airway or food pipe is affected. They may also help if you have cancer. 

Your provider may also refer you to a specialist, including:

• An ophthalmologist (an eye specialist) if they think you have signs of ocular MMP 
• An ENT (ear, nose, throat) specialist
• A dermatologist (a skincare specialist) 
• A dentist

How Is Mucous Membrane Pemphigoid Treated?

There is no cure for MMP yet, and lesions rarely go away without treatment. Even with appropriate treatment, they may come and go periodically.  

Treatment aims to control blistering, promote healing, and prevent complications. Before recommending specific treatment, your provider will discuss treatment options and their benefits/risks. 

Treatment can vary depending on the severity of the condition and include [4]:

Medications

For mild-to-moderate cases, primary treatment includes dapsone, methotrexate, or tetracyclines (with or without topical corticosteroids). 

If you have severe symptoms, your provider will likely recommend dapsone and oral or IV cyclophosphamide (with or without oral corticosteroids).

Wound Care

Wound care involves daily gentle cleaning, dressing, and topical medicines. 

Eye Care

Eye care involves lubricating eye drops and silicon plugs to help reduce dryness. 

Screening for Cancer

The risk of cancer may be higher in those with specific autoantibodies. In such cases, appropriate screening can help detect cancer early. 

Surgery

Your provider may recommend surgical procedures to:

• Prevent corneal scarring
• Open the airway
• Help with swallowing

Can IVIG Help Improve Mucous Membrane Pemphigoid?

According to a 2016 review, IVIG can benefit people who did not respond to or could not tolerate conventional therapy [5].

How IVIG works for MMP is not fully understood. Nonetheless, it is thought to remove causative antibodies from circulation. 

Mucous Membrane Pemphigoid: What’s the Outlook?

How MMP progresses is often hard to predict. To make matters worse, many individuals do not respond to conventional therapy. 

There have been reports of long-lasting remissions (periods without symptoms) in some individuals. However, in most cases, the symptoms reappear. 

Thus, regular monitoring and follow-ups are recommended. 

Luckily, there seems to be no connection between MMP and lifespan. 

What Is the Difference Between Pemphigus and Pemphigoid?

Both are blisters, but the pemphigus is fragile and opens easily. On the other hand, pemphigoid is stronger and does not break open easily. 

Living With Mucous Membrane Pemphigoid: Dietary and Activity Tips

• Avoid acidic foods, such as tomatoes, lemon, and orange juice. 
• Limit or avoid foods with hard surfaces, such as nuts, uncut fruits, and chips. 
• If you are on corticosteroid therapy, maintain adequate calcium and vitamin D levels through diet and supplements. Ask your doctor or provider to learn what is the optimum level for your condition. 
• Use contact lenses or dental plates carefully. 
• Minimize participation in contact sports. Instead, go for activities like swimming or water exercises. 
• Quit smoking and drink in moderation. Find help at smokefree.gov and MyHealthfinder. 
• Stick to your treatment plan. 

Five decades have passed since the term “common variable immunodeficiency” was first used in medicine. Still, many questions remain unanswered, and some answers are ambiguous. In this article, we will look at the latest evidence to answer some of the common questions about this condition.

What Is Common Variable Immunodeficiency?

Common variable immunodeficiency (CVID) is an umbrella term for immune disorders that cause low levels of infection-fighting proteins called immunoglobulins. As a result, people with CVID are more likely to experience frequent infections of the ears, sinuses, lungs, and digestive tract. 

Other names for this condition are:

• Common variable hypogammaglobulinemia
• Hypogammaglobulinemia, acquired
• Immunoglobulin deficiency, late-onset
• Idiopathic immunoglobulin deficiency
• Primary antibody deficiency
• Primary hypogammaglobulinemia

There are over 400 conditions that cause immunoglobulin deficiency. They are known as primary immunodeficiencies. Of them, CVID is relatively common, hence the name. 

However, CVID overall is rare, affecting fewer than 5,000 Americans. CVID is a long-term (chronic) condition that often requires lifelong treatment. 

What Causes Common Variable Immunodeficiency?

In about 90% of cases, researchers are not certain what exactly causes CVID. They believe both environmental and genetic factors may be responsible. 

Genetic changes (mutations) that cause CVID may be passed down from parents to children. In other cases, they may occur randomly or after exposure to triggers. Potential triggers include UV radiation and viral infections. 

Mutations in the genes that regulate B cell function are likely the culprit. B cells are specialized white blood cells; they produce immunoglobulins or antibodies. People with CVID have enough B cells, but these cells do not make enough antibodies. 

What Are the Risk Factors?

CVID can affect anyone, irrespective of age or sex. Nonetheless, it often affects adults, and most people are diagnosed between ages 20 and 50.

What Are the Symptoms of Common Variable Immunodeficiency?

Symptoms may be mild or severe and can vary from person to person. They can include:

• Nagging cough (sometimes, coughing up blood)
• Difficulty breathing
• Diarrhea
• Nausea
• Vomiting 
• Weight loss
• Ear infections
• Sinus infections
• Repeated lung infections, possibly leading to bronchitis or pneumonia  
• Unusual response to vaccines
• Abnormal liver
• Low platelet count
• Swollen joints
• Joint pain
• Enlarged spleen
• Swollen lymph nodes
• Red or purple spots on the skin

How Is Common Variable Immunodeficiency Diagnosed?

Your provider might suspect CVID if you have characteristic symptoms and risk factors, such as:

• Frequent ear, sinus, or lung infections
• Unusual response to vaccines
• A family history of the condition

To confirm the diagnosis, your doctor will order blood tests. These tests will help your provider determine antibody levels and how well your body responds to a vaccine. 

If your provider is sure you have CVID, they will recommend yearly tests to detect complications. Complications include autoimmune disorders, cancers, and lung problems. Tests may include:

• Blood tests
• Imaging tests

How Is CVID Treated?

No cure for CVID exists; however, there are several treatments to keep your condition in check. 
Once the diagnosis is confirmed, your provider will likely give you immunoglobulin (IgG) replacement therapy. It contains antibodies obtained from healthy donors. 
There are two ways you can receive donor-derived antibodies: 

1. Intravenous immunoglobulin (IVIG): IVIG is administered into a vein through a needle. The usual dose is 400 – 600 mg/kg every 2 to 4 weeks. 
2. Subcutaneous immunoglobulin (SCIG): SCIG is given as a shot under your skin. The usual dose is 160 mg/kg once a week. 

Most people respond well to IgG replacement therapy. It is highly effective in reducing the recurrence of infections and joint problems. Likewise, IgG therapy reduces the severity of complications, such as autoimmune disorders. People often need treatment for their lifetime. 
During the therapy, your healthcare provider will monitor you for side effects. Common side effects include backache, nausea, vomiting, chills, fever, muscle pain, and fatigue. To reduce the odds of such reactions, premedication may be necessary. 
Your provider may prescribe antibiotics to treat infections. In some cases, antibiotics may be used regularly to prevent infections. 
Other treatments are specific to the system involved. For example, vitamin B12 injections may help someone with digestive problems leading to B12 deficiency. Likewise, those with breathing difficulty may benefit from inhaled corticosteroids and similar medications. 
If you have a low platelet count, it is best to avoid aspirin. Moreover, people with CVID should not receive live virus vaccines. Examples include the chickenpox vaccine and the MMR vaccine.
 

Treatment Can Be Challenging in People With COVID

COVID-19 may be more severe in people with immunodeficiencies like CVID, though a clear association is lacking. Most people with CVID at risk of COVID-19 are treated with convalescent plasma or IVIG. 
 

What Are the Complications of CVID?

CVID can make you more likely to get:

• Autoimmune disorders. These disorders cause your immune system to destroy the body’s healthy tissues. People with CVID are at higher risk of developing immune thrombocytopenia or autoimmune hemolytic anemia.
• Bronchiectasis. This occurs when there is irreversible damage to the lungs due to repeated infections.
• Cancers. Blood cancer and stomach cancer occur more frequently in people with CVID. 

Can Women With CVID Get Pregnant?

Thanks to IVIG, women with CVID can get pregnant without any additional risks. However, they should receive periodic IVIG therapy with customized doses to reach the desired IgG levels in the newborn [1].
 

What Is the Life Expectancy of a Person With CVID?

With timely diagnosis and appropriate treatment, most people live well into adulthood. The key factors that affect life expectancy are:

• Severity of autoimmune disorders
• Recurrent infections, particularly those affecting the lungs
• Development of cancers

IVIG and antibiotic therapy have dramatically reduced deaths due to bacterial infections. Most deaths occur due to noninfectious complications, such as cancers. 
 

What Is the Difference Between CVID and IgG Deficiency?

CVID causes low levels of almost all immunoglobulin classes, whereas IgG deficiency is when you have low levels of immunoglobulin G (IgG). The five major immunoglobulin classes are IgA, IgG, IgM, IgD, and IgE. 
 

What Infections Are Common in CVID?

The most frequent infections in CVID are those involving the ears, sinuses, and respiratory tract. 
 

Five decades have passed since the term “common variable immunodeficiency” was first used in medicine. Still, many questions remain unanswered, and some answers are ambiguous. In this article, we will look at the latest evidence to answer some of the common questions about this condition.