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Mucous Membrane Pemphigoid

What is mucous membrane pemphigoid? 

This autoimmune blistering disorder is marked by subepithelial bullae (blistering lesions) on various mucous membranes throughout the body. Mucous membrane pemphigoid, or MMP, happens when basement membranes are targeted and attacked by autoantibodies. More often, mucous membrane pemphigoid occurs on sensitive mucous membrane areas – the eyes, larynx, esophagus, genitourinary tract, anus, and inside areas of the mouth, including gums, cheeks, and tongue.  

MMP is often referred to as cicatricial pemphigoid or oral pemphigoid and can even present as a rare variant called Brunsting Perry cicatricial pemphigoid. This variety is marked by recurrent blisters appearing on the head and neck in  urticarial plaques. Often, the blisters burst, creating scars. 

What causes mucous membrane pemphigoid? 

Autoimmune disorders, like mucous membrane pemphigoid, make it difficult to nail down causation. The body’s natural defenses – antibodies and immunoglobulins – don’t work as they should in attacking foreign invaders, but rather target normal, healthy tissue for one reason or another. When autoantibodies (antibodies that attack healthy tissue) go after autoantigens (healthy tissue and cells),  in the body’s mucosal membranes, create blistering lesions. 

As this attack begins in the basement membrane zone (BMZ) of the epithelium (the surface cells that cover the body and line internal organs).  Think of the basement membrane zone as a glue that connects the epidermis onto all of the tissue underneath. Something in the immune system tells autoantibodies to go after this “glue” and destroy it, causing the outermost layer to disconnect in areas, creating blisters. 

Experts believe that some cases of MMP occur in individuals who are genetically predispositioned. In other cases, factors like immunological and environmental factors can play a role in the occurrence of MMP. A small portion of cases are brought on my prescription drugs or surgeries, including cataract removal. 

Signs of MMP 

Obvious blisters are the main sign of MMP and these lesions present in a variety of ways and in different places depending on the individual. Here are general signs of mucous membrane pemphigoid: 

  • Red, blistering lesions
  • Obvious blisters on ocular region
  • Conjunctivitis 
  • Scars once lesions subside
  • Blisters in the mouth
  • Tender, bleeding gums that slough off in shreds 
  • Difficulty swallowing and hoarseness

Complications from Mucous Membrane Pemphigoid

While certain complications can be obvious – like pain, irritation, and scarring – other secondary problems can arise as MMP progresses. 

  • Dysphagia. Trouble swallowing while blistering lesions are forming. If serious scarring occurs, the individual may have permanent hoarseness, suffer from malnutrition, and other complications that result from stenosis (narrowing) of the esophagus and larynx. 
  • Asphyxiation. In extreme cases, scarring in the esophagus and larynx can occur prohibiting normal air flow and eventually causing the individual to suffocate.
  • Glaucoma. Grittiness, conjunctivitis, and pain can eventually lead to pressure in the eye, making reading, working, and general life difficult. 
  • Entropion. An inward turning of the eyelid can happen as MMP takes over the mucous membrane of the eye. 
  • Trichiasis. An inward turning of the eyelashes that then irritates the eye constantly. 
  • Blindness. In extreme cases, MMP can cloud the cornea and cause cataracts, other ocular issues, then blindness.
  • Alopecia. If MMP covers the head, neck, and scalp, the eventual scarring can block hair follicles and result in blindness. 
  • Hyperpigmentation. Scarring on the skin can lead to discoloration and skin that was affected may never return to its original pigment. This is more common in individuals with darker skin.
  • Bullous pemphigoid. Most common in the elderly, bullous pemphigoid is a chronic skin condition marked by large blisters on seemingly regular-looking skin. BP can also crop up around traumatized areas around cuts and other wounds. Unlike MMP, BP does not typically attack mucous membranes and usually heals on its own. Extreme irritation occurs and individuals often seek professional treatment to deal with symptoms. 
  • Epidermolysis Bullosa Acquisita (EBA). Another autoimmune disorder occurs in conjunction with skin trauma on the elbows, knees, pelvis, buttocks, and scalp. Blisters appear in these areas and leave scars when they heal. 
  • Pemphigus Vulgaris (PV). Marked by oral lesions, PV is difficult to diagnose because these lesions appear and break quickly. Blisters spread slowly to other areas – oftentimes existing as oral lesions only for 1-3 years.
  • Lichen Planus (LP). This common skin disorder appears as white, lacy lines inside the cheek. It can also present as its erosive form, creating red, bleeding gums and sores. LP on the skin appears as purplish, irregularly shaped bumps on the wrists, forearms, and ankles.
  • Brunsting-Perry Syndrome. This antiquated medical term refers to localized pemphigoid in which lesions affect the head, neck, and other parts of the skin but not the mucosal membranes. Sometimes, these severe blisters are triggered by trauma. 

Treatment 

Dealing with MMP is patient specific and the treatments vary greatly depending on the severity and the area of the body affected. After diagnosing it visually or through a biopsy, treatment can be created for the patient’s specific occurrence. 

Treatments focus on healing or minimizing the symptoms and often involve physicians from multiple disciplines. 

  • Corticosteroids. Topical creams containing corticosteroids (fluocinonide) suppress inflammation and can alleviate itching, burning, and pain when MMP affects the oral cavity, throat, and skin. 
  • Good oral hygiene. Keeping the area clean and germ free can boost the effectiveness of oral corticosteroid creams. When using potent gels like betamethasone dipropionate and clobetasol, it’s essential to maintain good oral hygiene. 
  • Antibiotics. Dapsone and other sulfa-based medications help when MMP is mild. Before beginning a course of antibiotics for MMP, blood tests are needed. Some individuals find success with tetracycline and nicotinamide but any antibiotic course needs to be closely monitored by a doctor. 
  • Immunosuppressive Drugs. Mycophenolate mofetil, cyclophosphamide, or azathioprine can help quell the intense immune system response that causes MMP in the first place. 
  • IV drug treatments. Taking drugs like rituximab or immunoglobulin via IV may help severe cases of MMP but this course of treatment has serious side effects and is often implemented when nothing else works. 
  • Surgery. Sometimes a biopsy is needed to identify the specific variant. Additionally, surgery is necessary in the widening of the airway or reversal of inverted eyelids can be necessary with MMP. 

Next Steps

If you are experiencing any signs of symptoms of mucous membrane pemphigoid, it’s vital you seek a professional diagnosis. If treated early, complications from MMP can be mitigated. However, since the autoimmune disorder presents in a wide variety of ways, diagnosing it correctly and quickly is vital. 

Because there are often associated diseases and symptoms that affect various areas of the body, doctors from a variety of disciplines should be consulted. A Lake Nona Dermatologist can act as your first line of defense when dealing with MMP and related autoimmune skin disorders. 

UCF Health’s patient portal can help you locate medical professionals, health-related resources, and COVID-19 updates for patients. Use our online scheduling tool to book an initial appointment. Since MMP can’t be cured, it’s crucial to seek professional treatment to mitigate complications and reduce life-hindering symptoms.