Cicatricial pemphigoid


Cicatricial pemphigoid (CP) refers to a group of rare chronic autoimmune blistering diseases that predominately affects the mucous membranes, including the conjunctiva, and occasionally the skin.


Usually beginning as a chronic conjunctivitis, the condition progresses to symblepharon (adhesion between the tarsal and bulbar conjunctiva); trichiasis (in-turning eyelashes); keratoconjunctivitis sicca; corneal neovascularization, opacification, and keratinization; and conjunctival shrinkage and keratinization. Chronic corneal epithelial defects can lead to secondary bacterial ulceration, scarring, and blindness. Oral mucous membrane involvement with ulceration and scarring is common, but skin involvement, characterized by scarring bullae and erythematous plaques, is uncommon.


Cicatricial pemphigoid is an autoimmune blistering disease, which basically means that an individual's immune systems starts reacting against his or her own tissue. In this particular instance autoantibodies react with proteins found in mucous membranes and skin tissue resulting in blistering lesions. The binding site appears to be within the anchoring filaments that help the epidermis (outside layer of skin) stick to the dermis (inner layer of skin).


* Bullous pemphigoid * Epidermolysis bullosa * Erythema multiforme * Linear IgA dermatosis * Pemphigus * Desquamative gingivitis


The disease is chronic, progressive and difficult to treat. There may be exacerbations and remissions with waning disease activity. The risk of malignancy with particularly antiepilgrin CP is significant.


The primary aim of treatment is to stop blister formation, promote healing and prevent scarring. Cicatricial pemphigoid is a particularly difficult disease to treat as it can affect so many different parts of the body. Usually a team of medical specialists are required for overall care. Medical treatment is very much dictated by the organs involved.