Cicatricial pemphigoid is a chronic, bilateral, progressive scarring and shrinkage of the conjunctiva with opacification of the cornea. Early symptoms are hyperemia, discomfort, itching, and discharge; progression leads to eyelid and corneal damage and sometimes blindness. Diagnosis is sometimes confirmed by biopsy. Treatment often requires systemic immunosuppression.
Cicatricial pemphigoid is an autoimmune disease in which binding of anticonjunctival basement membrane antibodies results in conjunctival inflammation. It is unrelated to bullous pemphigoid.
Symptoms and Signs
Usually beginning as a chronic conjunctivitis with nonspecific hyperemia without discharge in certain quadrants, the condition progresses to symblephara (adhesions 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.
Diagnosis is suspected clinically in patients with conjunctival scarring plus corneal changes, symblephara, or both. The differential diagnosis of progressive conjunctival scarring includes previous radiation exposure and atopic disease. Therefore, the clinical diagnosis of cicatricial pemphigoid is made when there is progression of a symblepharon without a history of local radiation or severe perennial allergic conjunctivitis. Diagnosis can be confirmed by conjunctival biopsy showing antibody deposition on the basement membrane. A negative biopsy does not rule out the diagnosis.
Tear substitutes and epilation, cryoepilation, or electroepilation of the in-turning eyelashes may increase patient comfort and reduce the risk of ocular infection, secondary corneal scarring, and decreased vision. For progressive trichiasis, conjunctival scarring, or corneal opacification or for nonhealing corneal epithelial defects, systemic immunosuppression (eg, with dapsone or cyclophosphamide) is indicated.
Last full review/revision October 2012 by Melvin I. Roat, MD, FACS