Mucous Membrane Pemphigoid
Mucous membrane pemphigoid (MMP) is a chronic, autoimmune disorder characterized by blistering lesions that primarily affect the various mucous membranes of the body, but also affects the skin (MMP is now the preferred term for lesions only involving the mucosa). It is also known as Cicatricial Pemphigoid (CP), as it is often scarring.
The mucous membranes of the mouth and eyes are most often affected, but those of the nose, throat, genitalia, and anus may also be affected. The symptoms of MMP vary among affected individuals depending upon the specific site(s) involved and the progression of the disease. Disease onset is usually between 40 and 70 years, and oral lesions are seen as the initial manifestation of the disease in 90% of cases. Blistering lesions eventually heal, sometimes with scarring. Scarring from lesions in the eyes or throat may potentially lead to serious complications.
Pemphigus vulgaris (PV) is the most common form of pemphigus (~80%). It is an acquired autoimmune disease in which antibodies target desmosomal proteins, leading to intraepithelial, mucocutaneous blistering.
The oral mucosa is often the first site of involvement. Typically, patients will have had multiple oral ulcers that have persisted for weeks or months. PV affects the oral mucosa in nearly all cases. The oral mucosa is the first affected site in 80% of cases, and it is the only affected site in 24% of cases.
A minority of patients only experience skin lesions. Skin involved is typically the upper chest, back, scalp, and face, but lesions can occur on any part of the body. The condition progresses over weeks to months.
Note that the skin-dominant form manifests the Dsg1 autoantibody profile. The mucosal dominant form manifests the Dsg3 autoantibody profile. A mixed pattern of skin and mucosa manifests a mixed Dsg1 and Dsg3 autoantibody profile.