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Cicatricial pemphigoid

Evidence: Antibody

Names 

Cicatricial pemphigoid
MMP
Mucous membrane pemphigoid
Ocular pemphigoid
Scarring pemphigoid

Description

Cicatricial pemphigoid is a rare autoimmune disease, discovered in 2002, that causes blistering of the mucous membranes. This blistering is due to autoantibodies binding to anchoring filaments of the epidermis.  A common mucous membrane that is affected is the conjunctiva of the eye.  Other areas that can be affected include the skin, nose, mouth, and genitals.

Cicatricial pemphigoid is usually not chronic, and most patients symptoms disappear within 3 years.

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Prevalence

Incidence, US cases: 6,000 - 40,000 annually [Hübner 2016] Most cases last 3 years or less.

Typical Age of onset

60-70 years

Symptoms

  • Eye sensitivity

  • Conjunctivitis

  • Lesions that lead to scar tissue

  • Nutritional deficiencies and weight loss

  • Blisters

  • Nose bleeds

  • Pain passing urine

  • Adhesions causing narrowing of the vaginal opening