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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 both eyelid and corneal damage and sometimes blindness. Diagnosis may be confirmed by biopsy, but this is often not necessary. Treatment may require 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, 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.

Diagnosis

• Unexplained symblepharon or biopsy findings

Diagnosis is suspected clinically by the presence of conjunctival scarring plus corneal changes, symblepharon, or both. The differential diagnosis of progressive conjunctival scarring includes postradiation and atopic disease. Therefore, the clinical diagnosis of cicatricial pemphigoid is made when there is progression of 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.

Cicatricial Pemphigoid

Treatment

• Epilation of in-turning lashes
• Sometimes systemic immunosuppression

Tear substitutes and epilation, cryoepilation, or electroepilation of the in-turning eyelashes may increase patient comfort and reduce the risk of ocular infection and secondary scarring. For progressive scarring or corneal opacification or for nonhealing corneal epithelial defects, systemic immunosuppression with dapsone Some Trade Names
ACZONE
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or cyclophosphamide Some Trade Names
CYTOXAN
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is indicated.

Last full review/revision June 2008 by Mitchell H. Friedlaender, MD

Content last modified June 2008