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Anti-BP180 (type XVII collagen)
Anti-BP230
Anti-laminin-332
Sometimes IgA autoantibodies (in rare variants)
Mucous membranes (mouth, eyes, genitals, throat)
Occasionally the skin
Cicatricial Pemphigoid is a rare chronic autoimmune disease that causes blistering and scarring of mucous membranes, particularly in the mouth, eyes, throat, nose, and genitals. In some cases, the skin may also be affected.
Over time, repeated blistering and inflammation can lead to permanent scarring—especially in the eyes, where it may cause vision loss if untreated.
Painful mouth sores or ulcers
Red, irritated eyes that may scar or cause vision problems
Blisters on the gums that rupture easily
Blistering or erosions on genitals or anus
Nasal crusting or nosebleeds
Difficulty swallowing or hoarseness (when the esophagus or larynx is involved)
In advanced cases, scarring of eyelids, vision loss, or airway narrowing
Cicatricial Pemphigoid is an autoimmune disorder, meaning the immune system attacks the body’s own tissues. In this case, it targets proteins in the basement membrane that anchor skin and mucous membranes.
Autoantibodies attack:
BP180 (type XVII collagen)
BP230
Laminin-332 (associated with more severe disease)
Biopsy of affected mucosa, showing subepithelial blisters
Direct immunofluorescence to detect IgG or C3 deposits in tissue
Blood tests for circulating autoantibodies (less reliable than biopsy)
Ophthalmologic, ENT, and dental evaluations to assess the extent of disease
Topical corticosteroids for mild disease (e.g., mouth ulcers)
Systemic immunosuppressants for moderate to severe disease:
Prednisone, azathioprine, mycophenolate mofetil
Dapsone (especially helpful for oral involvement)
Rituximab or IVIG for severe or treatment-resistant cases
Lubricating eye drops or specialized eye care if ocular disease is present
Regular monitoring for scarring and airway involvement
Early treatment improves outcomes and reduces scarring
Disease course is chronic and often relapsing
May require long-term immunosuppressive therapy
Eye involvement can lead to blindness without prompt care
Multidisciplinary approach improves quality of life
Rare: estimated at 1 to 5 per million people
Typically affects adults over 60, but can occur at any age
Slight female predominance
Rashid KA, et al. (2019). Mucous membrane pemphigoid: Pathogenesis, diagnosis, and multidisciplinary care. J Am Acad Dermatol.
National Organization for Rare Disorders (NORD). Cicatricial Pemphigoid.
https://rarediseases.org/rare-diseases/cicatricial-pemphigoid/
Chan LS, et al. (2002). The role of autoantibodies in mucous membrane pemphigoid. Dermatol Clin.
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