What is Benign mucosal pemphigoid (Mucous membrane pemphigoid)

Cicatricial pemphigoid (also known as benign mucosal pemphigoid, mucous membrane pemphigoid, or benign mucous membrane pemphigoid) is a rare chronic autoimmune blistering disease characterized by erosive skin lesions of the mucous membranes and skin that results in scarring of at least some sites of involvement. The autoimmune reaction most commonly affects the mouth, causing lesions in the gums, but it can also affect areas of mucous membrane elsewhere in the body, such as the sinuses, genitals and anus. When the cornea of the eye is affected, repeated scarring may result in blindness. The management depends upon the severity of the condition. Simple measures that can be taken include avoidance of hard, sharp or rough foods, and taking care when eating. Good oral hygiene is also usually advised, and professional oral hygiene measures such as periodontal scaling.

Mucous membrane pemphigoid is a rare, chronic, blistering and scarring disease that affects the oral and ocular mucosa. Other mucosal sites that might be affected include the nasopharnyx, larynx, genitalia, rectum, and esophagus. The condition usually begins in late adulthood (e.g. 50’s or 60’s), affects more women than men, and has a variable prognosis.[1] Scarring of the affected mucosa of the eye may lead to blindness and tends to be the most feared complication.[2] A combination of environmental and genetic factors appear to play a role in the susceptibility of developing cicatricial pemphigoid.[1] Although the specific causes of this condition have not been identified, it is considered an autoimmune disease that is characterized by the production of autoantibodies against basement membrane zone antigens such as BP180, BP230, and laminin 5.[2][3] Treatment is dependent on the person’s specific symptoms.

This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD). https://rarediseases.info.nih.gov/diseases/5913/mucous-membrane-pemphigoid