Mucous Membrane Pemphigoid

Further information can be obtained from the British Association of Dermatologists website at http://www.bad.org.uk/for-the-public

Mucous Membrane Pemphigoid

This may also be called:-

  • Benign Mucous Membrane Pemphigoid
  • Cicatricial Pemphigoid
  • Ocular Cicatricial Pemphigoid (when the eyes are affected)
  • Oral Pemphigoid

In May 1999, there was an International consensus on streamlining medical communication and agreeing a naming convention of Mucous Membrane Pemphigoid.

Mucous Membrane Pemphigoid (MMP) is an autoimmune blistering disorder that is characterized by subepithelial blisters. Various basement membrane zone components have been identified as targets of autoantibodies in MMP and the disease is characterised by Dermatologists based on these components.

Diagnosis of MMP is often difficult because patients present their symptoms in many different ways. Mucous membranes that may be involved include the mouth, eyes, nose, pharynx, larynx, esophagus, genitourinary tract, and anus. The skin is involved in approximately one-quarter of patients but is often limited to the head, neck, and upper torso regions.

A multidisciplinary approach is essential in the management of MMP, and the first specialist to see the patient may be a dentist, an ophthamologist, a gastro-enterologist, a Gynaecologist, a Dermatologist or other medical expert. Early recognition of this disorder and treatment is vital, however, as the resulting scarring may increase disease-related complications and impact. The choice of agents for treatment of MMP is based upon the sites of involvement, clinical severity, and disease progression. For more severe disease, or with rapid progression, systemic corticosteroids are the agents of choice for initial treatment, combined with steroid-sparing agents for long-term maintenance. There are few large controlled studies due to the rarity of the disease.

What are the symptoms of Mucous Membrane Pemphigoid?

MMP can develop in many ways. Mucous Membrane Pemphigoid causes blisters or ulcers that may appear in the oral cavity, conjunctiva, nasopharynx, larynx, esophagus, genitourinary tract, and anus. The mucosa in the mouth is most commonly involved, followed by the eyes.

Patients may present with only one site involved, or any combination of sites. In the mouth, the most common site of involvement, vesicles, erosions, desquamative gingivitis, and occasional scarring may appear.

Scarring may continue undetected by the patient, and, consequently, regular monitoring is important.

MMP in the eyes may create complications from conjunctival erosions with subsequent scarring and progressive cicatrization with foreshortening of the fornices The lining of the lids may fuse with the conjunctiva, resulting in scar bands between the lid and globe called symblepharon. The scarring of the conjunctiva can also results in dry eye and disruption of the corneal epithelium

Eyelids may also turn in at the lid margin resulting from the scarring process of the conjunctiva. This results in the eyes being scratched by misdirected eyelashes, further compromising the corneal integrity and contributing to corneal. Blindness and even loss of the globe due to perforation and endophthalmitis may occur.

Anogenital involvement presents with blisters and erosions that may result in scarring. Severe vulvar scarring and phimosis (narrowing) have been reported.

Life-threatening complications may result from involvement of the larynx, esophagus, and rarely the lower airway.

Long term, the scarring in the nose and larynx can create problems with crusting and a hoarse voice. Hearing can also be affected by the blockage of the Eustachian tube and there may be a loss of the sense of smell.

How is Mucous Membrane Pemphigoid managed?

A multidisciplinary approach is essential in the management of MMP. Early recognition and treatment may decrease disease-related complications. In a patient who presents with involvement of one site, a thorough review of symptoms highlighting other potential areas of involvement should be obtained. The choice of agents for treatment of MMP is based upon the sites of involvement, clinical severity, and disease progression.

The First International Consensus on MMP recommended dividing patients into “low-risk” and “high-risk” groups based upon the site(s) of involvement, with “low-risk” patients defined as having only oral mucosal or oral and skin involvement. “High-risk” patients were defined as having involvement of the ocular, genital, nasopharyngeal, esophageal, and/or laryngeal mucosae, and require more aggressive treatment.

A good description, albeit old, of the treatment of MMP can be found at http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2500254/