Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid, is a heterogeneous group of chronic autoimmune blistering diseases caused by autoimmunity to various components of the basement membrane. To date, autoantibodies to over 10 different antigens in the basement membrane zone have been identified: bullous pemphigoid antigen 1 (BP230), bullous pemphigoid antigen 2 (BP180, type XVII collagen), laminin-5 (laminin-332), laminin-6 (laminin-311), type VII collagen, integrin beta 4 subunit of α6β4 integrin, 45-kD unknown epithelial protein, 168-kD unknown epithelial protein, 120-kD unknown epithelial protein, and uncein. Patients with autoantibodies to β4 integrin present predominantly with ocular disease.

MMP is almost twice as common in females as it is in males, and it is seen most often in older individuals. MMP affects the mucous membranes and, less commonly, the skin. The mouth is involved most frequently (ie, painful erosions and ulcers), followed by the conjunctiva (corneal inflammation and scarring, conjunctiva inflammation, trichiasis, ectropion, symblepharon, ankyloblepharon, and blindness).

Skin, nasal, laryngeal, pharyngeal, and esophageal mucosal surfaces can also be affected, leading to epistaxis, hoarseness, or dysphagia, respectively. Anogenital involvement in women may present with vulvovaginal or perianal blisters, erosions, and scarring.

Cutaneous disease, when present, most frequently accompanies mucous membrane disease. Occasionally, cutaneous blistering and scarring dominate the clinical picture (so-called Brunsting-Perry variant).

In a 2022 study, malignancies, especially solid organ tumors, were reported in up to 13.8% of patients. These include lung carcinoma, breast cancer, endometrial cancer, vulvar carcinoma, and non-Hodgkin lymphoma. The malignancy rate was higher when autoantibodies against laminin-332 were found.