Mucous Membrane Pemphigoid
This page covers the following:
Introduction
Mucous Membrane Pemphigoid (MMP) can affect the skin, but more commonly it causes blisters in the mucous membranes (mouth, nose, throat, genital areas and anus). MMP also affects the eyes and, in serious and untreated cases, can result in blindness. The blisters can leave scars which causes longer term damage, in fact MMP used to be called Cicatricial Pemphigoid (scarring blisters). When the eyes are involved, it is usually called Ocular MMP or OCP (Ocular Cicatricial Pemphigoid).
MMP can involve one or more of the sites mentioned above, but often starts in the mouth with painful ulceration. Scarring may continue undetected by the patient, and, consequently, regular monitoring is important. 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 long will my mucous membrane pemphigoid last?
MMP is a chronic disease. There is no cure, but remission is possible. However, MMP is difficult to treat successfully, and the effects of the scarring can yield long term damage. An early diagnosis is a great advantage.
How is mucous membrane pemphigoid treated?
Due to the range of sites which can be affected by MMP, a multidisciplinary approach is essential in the management of the disease. 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 sought.
The choice of treatment for MMP is based upon the sites of involvement, clinical severity, and disease progression. In 2002, 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, oesophageal, and/or laryngeal mucosae, and required more aggressive treatment.
What treatments might I be offered to reduce the impact of mucous membrane pemphigoid?
Targeted topical steroid treatment to manage specific body sites are an important part of management. e.g. steroid mouthwash to treat oral pemphigoid and steroid ointments to treat pemphigoid affecting the skin. Oral steroids are also often prescribed initially especially for more severe forms of MMP. Once the disease is under control, then an alternative drug will be used to replace the steroids. These are called “steroid sparing drugs” as they have less severe long-term effects than steroids. Steroid sparing drugs can still have their own side effects and it may take a while to find an appropriate treatment. A biologic treatment called rituximab has been shown to be quite effective in bringing MMP into remission. A special funding request would usually need to be submitted for rituximab to be considered if other treatments, such as dapsone, and immunosuppressants, such as mycophenolate and azathioprine, have been ineffective, or if there is evidence of severe ocular pemphigoid. In Wales, the request may be submitted earlier.
Where can I find out more information?
For further information, we recommend the British Association of Dermatology Patient Leaflet, and the New Zealand DermNet website. A paper entitled "Definitions and outcome measures for Mucous Membrane Pemphigoid: Recommendations of an international panel of experts" can be found here. Also, please see this publication describing the First International Consensus on MMP.