Ocular mucous membrane pemphigoid is an autoimmune disease Autoimmune Disorders In autoimmune disorders, the immune system produces antibodies to an endogenous antigen (autoantigen). The following hypersensitivity reactions may be involved: Type II: Antibody-coated cells... read more in which binding of anticonjunctival basement membrane antibodies results in conjunctival inflammation. It is unrelated to bullous pemphigoid Bullous Pemphigoid Bullous pemphigoid is a chronic autoimmune skin disorder resulting in generalized, pruritic, bullous lesions in older patients. Mucous membrane involvement is rare. Diagnosis is by skin biopsy... read more .
(See also Mucous Membrane Pemphigoid Mucous Membrane Pemphigoid Mucous membrane pemphigoid is the designation given to a heterogeneous group of rare chronic autoimmune disorders that tend to cause waxing and waning bullous lesions of the mucous membranes... read more and Overview of Conjunctival and Scleral Disorders Overview of Conjunctival and Scleral Disorders The conjunctiva lines the back of the eyelids (palpebral or tarsal conjunctiva), crosses the space between the lid and the globe (forniceal conjunctiva), then folds back on itself as it spreads... read more .)
Symptoms and Signs
Usually beginning as a chronic conjunctivitis with nonspecific hyperemia without discharge in certain quadrants, ocular mucous membrane pemphigoid progresses as follows:
Conjunctival shrinkage with loss of the inferior fornix
Symblephara (adhesions between the tarsal and bulbar conjunctiva)
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.
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. 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.
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 immunosuppression (eg, with dapsone, methotrexate, mycophenolate mofetil, cyclophosphamide, intravenous immunoglobulin [IVIG], or rituximab) is indicated.
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, epilation of in-turning lashes, and often systemic immunosuppression.