Ocular mucous membrane pemphigoid is a chronic, bilateral, progressive scarring and shrinkage of the conjunctiva with opacification of the cornea. Early symptoms are hyperemia and irritation; progression leads to eyelid and corneal damage and sometimes blindness. Diagnosis is sometimes confirmed by biopsy. Treatment often requires systemic therapy with dapsone or immunosuppressants.
Ocular mucous membrane pemphigoid is an autoimmune disease in which binding of anticonjunctival basement membrane antibodies results in conjunctival inflammation. It is unrelated to bullous pemphigoid.
(See also Mucous Membrane Pemphigoid and Overview of Conjunctival and Scleral Disorders.)
Symptoms and Signs of Ocular Mucous Membrane Pemphigoid
Usually beginning as a chronic conjunctivitis with nonspecific hyperemia without discharge in certain quadrants, ocular mucous membrane pemphigoid progresses as follows:
Subconjunctival fibrosis
Conjunctival shrinkage with loss of the inferior fornix
Symblephara (adhesions between the tarsal and bulbar conjunctiva)
Trichiasis (in-turning eyelashes)
Corneal epithelial defects and bacterial corneal infection
Corneal neovascularization, opacification, keratinization, 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 of Ocular Mucous Membrane Pemphigoid
Unexplained symblephara or biopsy findings
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Diagnosis of ocular mucous membrane pemphigoid 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 linear antibody deposition on the basement membrane. A negative biopsy result does not rule out the diagnosis.
Treatment of Ocular Mucous Membrane Pemphigoid
Topical lubrication
Topical anti-inflammatory therapy (eg, corticosteroids, cyclosporine)
Epilation of in-turning lashes
Often systemic immunosuppression
In people with ocular mucous membrane pemphigoid, tear substitutes and epilation, cryoepilation, or electroepilation of the in-turning eyelashes may increase 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 therapy with dapsone or immunosuppressants (eg, methotrexate, mycophenolate mofetil, cyclophosphamide, intravenous immunoglobulin [IVIG], rituximab) is indicated. Patients with nonhealing epithelial defects may also benefit from amniotic membrane transplantation.
Key Points
Ocular mucous membrane pemphigoid is a chronic, autoimmune scarring of the conjunctiva with opacification of the cornea.
Findings include symblephara (adhesions between the tarsal and bulbar conjunctiva); trichiasis (in-turning eyelashes); keratoconjunctivitis sicca; corneal neovascularization, opacification, and keratinization; and conjunctival shrinkage and keratinization.
Diagnosis is usually by finding a progressive symblepharon in a patient without a history of local radiation or severe perennial allergic conjunctivitis.
Treatment can include tear substitutes, topical anti-inflammatory therapy, epilation of in-turning lashes, and often systemic therapy with dapsone or immunosuppressants.
