Cicatricial Pemphigoid

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.

Autoimmune disorder where the immune system targets proteins in the basement membrane that anchor skin and mucous membranes. Autoantibodies attack BP180 (type XVII collagen), BP230, and 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; 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.

Rare: estimated at 1 to 5 per million people. Typically affects adults over 60, but can occur at any age. Slight female predominance.