ABSTRACT
Mucous membrane pemphigoid (MMP) consists of a heterogeneous group of autoimmune subepidermal blistering disorders characterized by predominant involvement of mucous membranes and occasional involvement of skin. The oral cavity is the most common mucosa involved, followed by conjunctiva, nasopharynx, anogenital region, larynx, and esophagus. The disease is marked by bullae, erosions, and in the later phases, scarring. Scarring of the esophageal and laryngeal mucosa can lead to strictures, a life-threatening complication. Malignancies and autoimmune disorders are the two disease entities that have been reported to have possible associations with MMP. The diagnosis of MMP rests on a combination of clinical presentation, histopathology, direct immunofluorescence findings, and serology. The treatment goal is to avoid or delay scarring, hence avoiding life-threatening complications. Systemic therapy is usually advocated in high-risk patients or in low-risk patients with poor or no response to topical therapy. Multiple immunosuppressive anti-inflammatory medications have been used. Rituximab is a promising agent for MMP therapy, especially in severe treatment-refractory disease. Therapy has to be individualized in each patient based on the disease characteristics and comorbidities of the patient. A multidisciplinary approach is desirable to improve outcomes. Surgical interventions may be required for restoring function and improving quality of life.
