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Ocular Mucous Membrane Pemphigoid

(Mucous Membrane Pemphigoid, Ocular Mucous Membrane Pemphigoid, Cicatricial Pemphigoid; Ocular Cicatricial Pemphigoid; Benign Mucous Membrane Pemphigoid)

By

Zeba A. Syed

, MD, Wills Eye Hospital

Reviewed/Revised Apr 2023
View PATIENT EDUCATION
Topic Resources

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.

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:

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

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 Overview of Allergic and Atopic Disorders Allergic (including atopic) and other hypersensitivity disorders are inappropriate or exaggerated immune reactions to foreign antigens. Inappropriate immune reactions include those that are... read more . 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 Seasonal and perennial conjunctivitis Allergic conjunctivitis is an acute, intermittent, or chronic conjunctival inflammation usually caused by airborne allergens. Symptoms include itching, lacrimation, discharge, and conjunctival... read more Seasonal and perennial 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 dapsone or systemic immunosuppressive therapy.

Drugs Mentioned In This Article

Drug Name Select Trade
Aczone
Cequa, Gengraf , Neoral, Restasis, Sandimmune, SangCya, Verkazia, Vevye
Jylamvo, Otrexup, Rasuvo, RediTrex, Rheumatrex, Trexall, Xatmep
CellCept, Myfortic
Cyclophosphamide, Cytoxan, Neosar
RIABNI, Rituxan, RUXIENCE, truxima
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NOTE: This is the Professional Version. CONSUMERS: View Consumer Version
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